Sinonasal respiratory epithelial adenomatoid hamartomas: Series of 51 cases and literature review.

Lee JT, Garg R, Brunworth J, Keschner DB, Thompson LD.
Am J Rhinol Allergy. 2013 Jul;27(4):322-8.
BACKGROUND: Respiratory epithelial adenomatoid hamartomas (REAHs) are rare, benign glandular proliferations of the nasal cavity, paranasal sinuses, and nasopharynx. This study aimed to expand our understanding of this entity by presenting a series of REAHs combined with a review of the pertinent literature.
METHODS: A retrospective review was performed on all patients with a diagnosis of REAH from 2002 to 2011. Data were collected with respect to age, gender, clinical presentation, imaging, histopathology, treatment, and outcome. Because olfactory cleft expansion by imaging evaluation has been reported to suggest REAH, maximum olfactory cleft (MOCs) widths were also measured.
RESULTS: Fifty-one cases of REAH included 37 male (72.5%) and 14 female subjects (27.5%) with a mean age of 58.4 years. Headache, nasal obstruction, rhinorrhea, and hyposmia were the most common presenting symptoms. Although 35(68.6%) were associated with concurrent inflammatory pathology, 16 (31.4%) presented as isolated lesions of the nasal cavity. Enlargement of MOCs was evident on computed tomography, with mean MOCs of 8.64 and 9.4 mm, in the coronal/axial planes, respectively. There were no statistically significant differences between MOCs of isolated (7.96 mm) versus MOCs of associated (9.63 mm) lesions (p = 0.25). Forty-nine were treated with endoscopic resection without evidence of recurrence after a mean follow-up of 27.2 months.
CONCLUSION: REAHs are rare sinonasal lesions that may appear as localized, isolated masses or more diffuse when in conjunction with other inflammatory processes. Irrespective of clinical presentation, endoscopic removal appears to be curative. Differentiation from more aggressive lesions is paramount to avoid unnecessarily radical surgery for an otherwise benign process.
PubMed ID: 23883815
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Invited Review: An update on salivary gland pathology.

Müller S, Thompson LD.
Head Neck Pathol. 2013 Jul;7 Suppl 1:1-2.
FIRST PARAGRAPH: Over the past few decades, salivary gland tumor pathology has evolved. This includes recognition of newly defined entities as well as reclassification of other salivary gland tumors [1]. The development of genetic tests have shown that some salivary gland tumors have genetic abnormalities which are specific to a histologic type such as MECT1/ MAML2 gene fusion in mucoepidermoid carcinoma and PLAG1 or HMGA2 gene translocation in pleomorphic adenoma. Immunohistochemical studies have aided in both the diagnosis and prognosis of salivary gland tumors. High Ki67 is correlated with poor overall survival in mucoepidermoid carcinoma, acinic cell carcinoma and adenoid cystic carcinoma. High MUC1 in mucoepidermoid carcinoma is associated with higher grade and high recurrence while MUC4 is associated with a lower grade tumor and longer disease free survival. The finding of androgen receptors in salivary duct carcinoma has led to new therapies as these tumors are shown to be responsive to androgen deprivation therapy. Newly described salivary gland entities in the past 20 years include both benign (sclerosing polycystic adenosis, sialolipoma) and malignant (cribriform adenocarcinoma of the tongue, mammary analogue secretory carcinoma) tumors [2]. Newly recognized histologic variants of well-known salivary gland tumors have been reported, to include salivary-duct carcinoma, acinic cell carcinoma and epi-myoepithelial carcinoma.
PubMed ID: 23821215
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Endolymphatic sac tumor.

Thompson LD.
Ear Nose Throat J. 2013 Apr-May;92(4-5):184-8.
FIRST PARAGRAPH: Endolymphatic sac tumor (ELST) is a papillary epithelial neoplasm arising within the endolymphatic sac/duct that shows a high association with von Hippel-Lindau disease (VHL). There is usually a VHL tumor suppressor gene germline mutation with an autosomal dominant inheritance pattern. Approximately 1 in 35,000 to 40,000 people have VHL, of which approximately 10 to 15% have endolymphatic sac tumors. There is a wide age range at presentation, although most patients are between 30 and 40 years; there is no gender predilection.
PubMed ID: 23599098
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Mucocele: Retention and extravasation types.

Thompson LD.
Ear Nose Throat J. 2013 Mar;92(3):106-8.
FIRST PARAGRAPHS: The extravasation type is the most common mucocele, more common in children and young adults, with a peak in the second decade of life. The most common non-neoplastic lesion of salivary gland tissue is the mucocele (also called sialocele and ptyalocele). A mucocele is defined as the pooling of mucin in a cystic cavity. Two types of mucoceles are recognized: (1) the retention type, in which the mucin pooling is confined within a dilated excretory duct or cyst, and (2) the extravasation type, in which mucin is spilled into the connective tissues from a ruptured or traumatized salivary gland duct.
PubMed ID: 23532645
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Wegener granulomatosis.

Thompson LD.
Ear Nose Throat J. 2013 Jan;92(1):18-22.
FIRST PARAGRAPHS: Wegener granulomatosis presents clinically as ulcerative and crusted lesions with tissue destruction. Wegener granulomatosis (WG) is an idiopathic, nonneoplastic, aseptic, necrotizing disease characterized by vasculitis and destructive properties. In general, affected patients exhibit disease in the sinonasal tract, lungs, and kidney, in some cases metachronously. Patients may present with systemic or localized disease; patients with systemic disease are usually quite sick. Disease progression may be seen when localized disease becomes systemic, but many patients will remain with limited disease.
PubMed ID: 23354886
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Nodular fasciitis.

Thompson LD.
Ear Nose Throat J. 2002 Dec;81(12):830.
FIRST PARAGRAPH: Nodular fasciitis is generally regarded as a benign, reactive, tumor-like proliferation of myofibroblasts. It quite commonly occurs in the head and neck region in young patients shortly after they develop an enlarging mass (ulceration is infrequent). In some cases, nodular fasciitis is associated with antecedent trauma.
PubMed ID: 12516376
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Metastatic renal cell carcinoma to the thyroid gland: a clinicopathologic study of 36 cases.

Heffess CS, Wenig BM, Thompson LD.
Cancer. 2002 Nov 1;95(9):1869-78.
BACKGROUND: Clear cell tumors of the thyroid gland in general are uncommon. Metastatic renal cell carcinoma (RCC) to the thyroid gland is a rare occurrence but must be considered in the differential diagnosis of a thyroid gland clear cell neoplasm to prevent misclassification, potentially resulting in inappropriate clinical management.
METHODS: Thirty-six cases of metastatic RCC to the thyroid were retrospectively retrieved from the files of the Endocrine Registry of the Armed Forces Institute of Pathology.
RESULTS: The tumors occurred in 22 women and 14 men, ages 53-80 years (mean, 64.9 years). Symptoms were present for a mean of 13.0 months. The tumors generally affected a single lobe of the thyroid gland as a solitary mass (n = 30; 83%), measuring 1.0-15.0 cm in diameter (mean, 3.8 cm). Histologically, the tumors were composed of polygonal cells with clear cytoplasm, distinct cell membranes, and small compact eccentric nuclei within a rich vascular network. Diastase-sensitive, periodic acid-Schiff-positive material (n = 22; 61%) and/or Oil Red O-positive material (n = 5; 14%) were noted. Thyroglobulin immunohistochemistry was negative in the foci of metastatic RCC. Although the majority of the patients had documented previous evidence of an RCC (n = 23; 64%) as remotely as 21.8 years before the thyroid metastases (mean, 9.4 years), the metastatic tumor to the thyroid gland was the initial manifestation of RCC in 13 patients. The majority of patients (n = 23; 64%) died with disseminated disease (mean, 4.9 years), but 13 patients (36%) were alive or had died without evidence of disease (mean, 9.1 years).
CONCLUSIONS: In the presence of a clear cell tumor of the thyroid gland, the diagnostic considerations must include metastatic RCC. The light microscopic features may suggest this possibility and the diagnosis can be established by supplemental histochemical and immunohistochemical studies. Surgical treatment of the metastatic disease is suggested, as this may result in prolonged patient survival. Published 2002 by the American Cancer Society.
PubMed ID: 12404280
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Hemangioma of the parotid.

Thompson LD.
Ear Nose Throat J. 2002 Nov;81(11):769.
FIRST PARAGRAPH: Hemangiomas are benign tumors of endothelial cell origin (benign hemangioendotheliomas). They are the most common salivary gland tumor in children younger than 1 year of age, accounting for 90% of cases. Hemangiomas in adults are uncommon. The characteristic features of hemangiomas are the rapid enlargement of a unilateral (usually on the left), compressible, bluish mass shortly after birth, particularly in girls. Hemangiomas are not associated with any syndrome.
PubMed ID: 12472029
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Relapsing polychondritis.

Thompson LD.
Ear Nose Throat J. 2002 Oct;81(10):705.
FIRST PARAGRAPH: Regardless of age, patients with relapsing polychondritis (systemic chondromalacia or polychondropathy) have at least one of eight progressively degenerative changes: (1) recurrent bilateral auricular chondritis, (2) inflammatory polyarthritis, (3) nasal chondritis, (4) ocular inflammation, (5) tracheal chondritis, (6) laryngeal chondritis, (7) cochlear damage, and (8) vestibular damage. Relapsing polychondritis is associated with other immunologically mediated diseases, such as systemic lupus erythematosus, rheumatoid arthritis, and Sjögren’s syndrome.
PubMed ID: 12405090
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Subacute (de Quervain’s) thyroiditis.

Thompson LD, Heffess CS.
Ear Nose Throat J. 2002 Sep;81(9):623.
FIRST PARAGRAPH: Subacute (de Quervain’s) thyroiditis is a seasonal disorder that generally affects middle-aged women. It is characterized by neck pain and generalized malaise, fatigue, fever, and chills following an upper respiratory infection, usually of viral etiology. Thyroid gland palpation elicits exquisite tenderness of the firm gland, which can be involved either focally or diffusely. The stage the disease determines whether there is hyper- or hypothyroidism.
PubMed ID: 12353438
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Rhinoscleroma.

Thompson LD.
Ear Nose Throat J. 2002 Aug;81(8):506.
FIRST PARAGRAPH: Rhinoscleroma (‘hard nose’) is caused by Klebsiella rhinoscleromatis, a gram-negative encapsulated bacterium of low infectivity. The disease is uncommon in the United States; most cases are found in the Middle East (especially Egypt), in parts of Latin America, and in Eastern Europe. The disease process usually involves the nasal cavity and the nasopharynx, but it can also involve the larynx, trachea, bronchi, middle ear, and orbit.
PubMed ID: 12199166
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Chondrosarcoma of the larynx: a clinicopathologic study of 111 cases with a review of the literature.

Thompson LD, Gannon FH.
Am J Surg Pathol. 2002 Jul;26(7):836-51.
Chondrosarcomas of the larynx are rare tumors accounting for about 0.5% of all laryngeal primary tumors. A total of 111 laryngeal chondrosarcoma cases, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. There was a 3.6:1 male/female ratio of patients 25-91 years of age (mean, 64.4 years). Patients presented most frequently with hoarseness (n = 72 patients) present for a mean of 28.2 months. The majority of tumors involved the cricoid cartilage (n = 77) with a mean size of 3.5 cm. All tumors were invasive and malignant by radiology and/or histology (into bone within the ossified laryngeal cartilages in 52 tumors). Most tumors were low-grade lesions: grade 1 (n = 51), grade 2 (n = 54); there were six grade 3 tumors. An associated benign chondroma with (n = 41 tumors) or without ischemia (n = 24 tumors) was noted. All patients had surgery and five had radiation therapy. Wide excision or voice-sparing surgery was used in 73 patients, whereas 37 patients had a laryngectomy. Recurrences occurred in 20 (18%) patients, 10 of whom underwent salvage laryngectomy. At the last follow-up, 102 patients had no evidence of disease (alive or dead, mean 11.2 years) and five patients had evidence of disease (alive, one patient, 6.5 years; dead, four patients, mean 6.4 years). The six patients with high-grade chondrosarcoma were all without disease at the last follow-up (mean, 15.1 years). There was no difference in clinical outcome based on grade (p = 0.210), location (p = 0.078), or treatment (p = 0.607) but was worse for patients with a myxoid-type chondrosarcoma (p = 0.044). Primary laryngeal chondrosarcomas are typically low- to moderate-grade lesions involving the cricoid cartilage, frequently associated with a chondroma. They usually portend an excellent overall long-term prognosis with initial conservative voice-sparing surgery.
PubMed ID: 12131151
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Basaloid squamous cell carcinoma of the sinonasal tract.

Wieneke JA, Thompson LD, Wenig BM.
Cancer. 1999 Feb 15;85(4):841-54.
BACKGROUND: Basaloid squamous cell carcinoma (BSCC) is a high grade, aggressive variant of squamous cell carcinoma with a predilection for the larynx, hypopharynx, tonsils, and base of the tongue. To the authors’ knowledge, BSCC originating in the nasal cavity and paranasal sinuses rarely has been reported.
METHODS: Fourteen cases of BSCC involving the nasal cavity and paranasal sinuses were identified in the files of the Otolaryngic-Head and Neck Pathology Tumor Registry of the Armed Forces Institute of Pathology from 1975-1997. Clinical records and follow-up were available in all cases. Paraffin blocks were available for histochemical and immunohistochemical studies in all cases.
RESULTS: There were 7 females and 7 males, ages 32-86 years (median, 66.5 years; mean, 62 years). The patients presented primarily with a mass lesion and unilateral nasal obstruction. In nine patients the tumor was confined to the nasal cavity. In three patients the tumor involved the sinuses alone and in two patients the tumor involved the nasal cavity and paranasal sinuses. Histologically, the tumors were widely invasive with a variety of growth patterns, including lobular, solid, trabecular, cribriform, and fascicular. The neoplastic infiltrate included predominantly pleomorphic, basaloid-appearing cells with hyperchromatic nuclei, inconspicuous to prominent nucleoli, and a variable amount of eosinophilic to clear-appearing cytoplasm. Mitotic figures, including atypical forms, were readily apparent as was necrosis (individual cell and comedo-type). Foci of squamous differentiation were limited in extent but were found in all cases and included squamous whorls, individual cell keratinization, and intercellular bridges. Intraepithelial dysplasia, carcinoma in situ, or invasive squamous carcinoma was present in all cases. Other histologic features included intercellular stromal hyalinization and peripheral nuclear palisading. In two cases, neural-type rosettes were found. Immunoreactivity for a variety of epithelial markers including cytokeratin (AE1/AE3/LP34), CAM 5.2, 34betaE12, CK7, and epithelial membrane antigen was present in all cases. Variable reactivity was present with vimentin, actins (smooth muscle and muscle specific), neuron specific enolase, S-100 protein, glial fibrillary acidic protein, CK20, carcinoembryonic antigen, Leu7, and Ewing’s marker. Chromogranin, synaptophysin, neurofibrillary protein, leukocyte common antigen, HMB-45, desmin, and Epstein-Barr virus latent membrane protein were absent. Surgical resection was the treatment of choice. Eight patients had recurrent or persistent tumor and metastatic disease occurred in five patients. At last follow-up, 7 patients (50%) had died of disease with a median survival of 12 months from the time of diagnosis and 3 patients were alive with disease over periods ranging from 8 months-5 years. Of the 4 remaining patients, 2 were alive without disease at 1 month and 5 years, respectively, 1 patient was lost to follow-up with no evidence of tumor at 3 years, and 1 patient had died of unrelated causes with no evidence of disease.
CONCLUSIONS: Sinonasal BSCC is a histologically distinct variant of squamous cell carcinoma with pathologic features and aggressive biologic behavior similar to BSCC localized to more common mucosal sites of the upper aerodigestive tract.
PubMed ID: 10091761
Article Size: 2 MB
 

Mucinous cystic neoplasm (mucinous cystadenocarcinoma of low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases.

Thompson LD, Becker RC, Przygodzki RM, Adair CF, Heffess CS.
Am J Surg Pathol. 1999 Jan;23(1):1-16.
Mucinous cystic neoplasms (MCNs) of the pancreas are uncommon tumors. The classification and biologic potential of these neoplasms remain the subject of controversy. Attempts to classify these tumors in a similar manner to ovarian MCNs remains controversial, as even histologically benign-appearing pancreatic MCNs metastasize and are lethal. One hundred thirty cases of MCNs were identified in the files of the Endocrine Pathology Tumor Registry of the Armed Forces Institute of Pathology from the years 1979 to 1993. The pathologic features, including hematoxylin and eosin staining, histochemistry, immunohistochemistry (IHC), cell cycle analysis, and K-ras oncogene determination were reviewed. These findings were correlated with the clinical follow-up obtained in all cases. There were 130 women, aged 20-95 years (mean age at the outset, 44.6 years). The patients had vague abdominal pain, fullness, or abdominal masses. More than 95% of the tumors were in the pancreatic tail or body and were predominantly multilocular. The tumors ranged in size from 1.5 to 36 cm in greatest dimension, with the average tumor measuring >10 cm. A spectrum of histomorphologic changes were present within the same case and from case to case. A single layer of bland-appearing, sialomucin-producing columnar epithelium lining the cyst wall would abruptly change to a complex papillary architecture, with and without cytologic atypia, and with and without stromal invasion. Ovarian-type stroma was a characteristic and requisite feature. Focal sclerotic hyalinization of the stroma was noted. This ovarian-type stroma reacted with vimentin, smooth muscle actin, progesterone, or estrogen receptors by IHC analysis. There was no specific or unique epithelial IHC. K-ras mutations by sequence analysis were wild type in all 52 cases tested. Ninety percent of patients were alive or had died without evidence of disease (average follow-up 9.5 years), irrespective of histologic appearance; 3.8% were alive with recurrent disease (average 10 years after diagnosis); and 6.2% died of disseminated disease (average 2.5 years from diagnosis). Irrespective of the histologic appearance of the epithelial component, with or without stromal invasion, pancreatic MCNs should all be considered as mucinous cystadenocarcinomas of low-grade malignant potential. Pancreatic MCNs cannot be reliably or reproducibly separated into benign, borderline, or malignant categories.
PubMed ID: 9888699
Article Size: 4 MB
 

A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature.

Paal E, Thompson LD, Heffess CS.
Cancer. 1998 Jun 1;82(11):2150-8. — Erratum in: Cancer 1998 Aug 15;83(4):824.
BACKGROUND: Pancreatic lymphangiomas are rare benign tumors, of which only a few cases have been reported in the literature. In this study, the authors present a series of primary pancreatic lymphangiomas.
METHODS: Cases of nonepithelial pancreatic cystic tumors (lymphangiomas) diagnosed between 1966 and 1994 were retrieved from the Endocrine Pathology Registry of the Armed Forces Institute of Pathology. Histologic features (in 10 cases) as well as histochemical and immunohistochemical studies (in 6 cases) were reviewed. Long term patient follow-up data were obtained in 9 cases.
RESULTS: The patients included 8 females and 2 males ages 2-61 years (mean age, 28.9 years) at initial presentation. The tumors were circumscribed and occurred predominantly (in 6 of 10 cases) in the tail of the pancreas. The multicystic, serous, or chylous fluid-filled cystic tumors ranged from 3 to 20 cm (average, 12.7 cm) in greatest dimension. Histologically, the tumors consisted of multilocular cystic spaces of various sizes, lined by endothelial cells. The stroma contained smooth muscle and mature lymphocytes. Immunohistochemistry determined the endothelial lining cells to be factor VIII-R antigen and CD31 positive (in all cases tested) but usually CD34 negative. All patients for whom follow-up data were obtained (n=9) were alive without evidence of disease an average of 7.2 years after initial diagnosis.
CONCLUSIONS: Pancreatic lymphangiomas occur predominantly in females within a wide age range. Multilocular, fluid-filled cysts, with endothelial immunoreactivity for factor VIII-R antigen and CD31, are characteristic of these tumors. Complete surgical excision of these benign tumors resulted in excellent long term prognoses for all patients studied.
PubMed ID: 9610694
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The clinical importance of cystic squamous cell carcinomas in the neck: a study of 136 cases.

Thompson LD, Heffner DK.
Cancer. 1998 Mar 1;82(5):944-56.
BACKGROUND: Predominantly cystic squamous cell carcinomas in the neck often present without a clinically apparent primary and therefore are frequently considered to be of branchial cleft origin. It is the authors’ hypothesis that the anatomic site of the primary carcinoma that produced the neck metastasis can often be predicted on the basis of the histologic features.
METHODS: Cases of cystic squamous cell carcinoma in the neck diagnosed between 1971 and 1991 were retrieved from the Otorhinolaryngic Pathology Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed and patient follow-up was obtained and analyzed.
RESULTS: In cases wherein the primary site was discovered subsequently, 64% of the primaries were in the lingual or faucial tonsil. An additional 8% of cases were in nasopharyngeal tonsillar tissue. The cases that did not originate in Waldeyer’s tonsillar ring generally differed in histologic appearance from the tonsillar cases. The tonsillar primaries were discovered within an average of 12.4 months, but many were not discovered for years (up to 11 years). Most were small, indicating a slower growth of the primary than is usually expected for squamous cell carcinoma. Patients with such carcinomas had a much better prognosis than patients with metastatic squamous cell carcinomas of other upper airway mucosal sites.
CONCLUSIONS: In most cases of prominently cystic squamous cell carcinomas in the upper neck, the origin of the primary site will be in faucial or lingual tonsillar crypt epithelium. Knowledge of the probable site of origin allows for more tailored therapy in which the patients can be treated relatively conservatively with surgical excision and subsequent field-limited radiation therapy only, with 77% survival at 5 years. None of the cases reviewed in this study was a branchiogenic carcinoma.
PubMed ID: 9486586
Article Size: 2 MB
 

Thyroid papillary carcinoma of columnar cell type: a clinicopathologic study of 16 cases.

Wenig BM, Thompson LD, Adair CF, Shmookler B, Heffess CS.
Cancer. 1998 Feb 15;82(4):740-53. — Comment in: Cancer. 1998 Dec 1;83(11):2421-3.
BACKGROUND: Thyroid papillary carcinoma of columnar cell type is considered an uncommon histologic subtype of papillary carcinoma characterized by its morphologic features and purportedly aggressive biologic course.
METHODS: Sixteen cases of thyroid papillary carcinoma of columnar cell type were identified from the Endocrine Tumor Registry at the Armed Forces Institute of Pathology and the Washington Hospital Center. Clinical records and follow-up were available in all cases. Paraffin blocks were available for histochemical and immunohistochemical studies in 15 of the 16 cases.
RESULTS: Of the 16 cases reported, 13 patients were female and 3 were male. The ages ranged from 16-76 years (average, 47 years; median, 40 years). An asymptomatic neck mass was the most common clinical presenting symptom. Macroscopically, the tumors varied from circumscribed or encapsulated to infiltrative, ranging in size from 1.5-6.5 cm. Histologically, the tumors had diverse growth patterns, including papillary, solid, microfollicular, and cribriform. A common pattern was the presence of markedly elongated follicles arranged in parallel cords. Colloid-filled follicles could be found, at least focally, in all cases. The characteristic histologic appearance included the presence of elongated cells showing nuclear stratification. Other features included the presence of vacuolated-appearing cells, spindle-shaped cells, and squamoid nests. Limited areas in the tumors showed morphologic features typical of thyroid papillary carcinoma. In 14 of the cases, the tumor was encapsulated, showed limited invasive growth, or was a microscopic tumor. In two of the cases, there was extrathyroidal invasion. Immunohistochemical studies showed consistent reactivity with cytokeratin and vimentin; varied reactivity with thyroglobulin, epithelial membrane antigen, carcinoembryonic antigen, and LeuM1; and no reactivity with calcitonin or chromogranin. Treatment was by surgical resection; supplemental radioactive iodine therapy was administered in eight patients. Follow-up was available for all patients, 13 of whom (81%) were alive and free of disease or had died of unrelated causes over periods ranging from 2-11 years (average, 5.8 years). All 13 of these patients had tumor confined completely to the thyroid gland. One patient died 4 months after diagnosis secondary to sepsis. Two patients (17%) had aggressive biologic courses. In both patients there was extrathyroidal invasion. One of these patients died of metastatic disease to the lungs 3 years after diagnosis; the other was alive with bilateral pulmonary metastases 9 years after the diagnosis.
CONCLUSIONS: The findings of the current study indicate that thyroid papillary carcinoma of columnar cell type is a distinct morphologic type but not a distinct clinical type of thyroid papillary carcinoma. The biologic behavior of this tumor is predicated on clinical stage, with the presence or absence of extrathyroidal invasion being the single most important parameter. Treatment of patients with these tumors should be based on the clinical stage and not on the morphologic appearance.
PubMed ID: 9477108
Article Size: 3 MB
 

Primary ameloblastoma of the sinonasal tract: a clinicopathologic study of 24 cases.

Schafer DR, Thompson LD, Smith BC, Wenig BM.
Cancer. 1998 Feb 15;82(4):667-74.
BACKGROUND: Ameloblastomas are locally aggressive jaw tumors with a high propensity for recurrence and are believed to arise from the remnants of odontogenic epithelium. Extragnathic ameloblastomas are unusual and primary sinonasal tract origin is extraordinarily uncommon.
METHODS: Twenty-four cases of ameloblastoma confined to the sinonasal tract were retrieved from the Otorhinolaryngic-Head & Neck Pathology and Oral-Maxillofacial Pathology Tumor Registries of the Armed Forces Institute of Pathology between 1956 and 1996.
RESULTS: The patients included 5 females and 19 males with an age range of 43-81 years, with a mean age at presentation of 59.7 years. The patients presented with an enlarging mass in the maxillary sinus or nasal cavity (n = 24), sinusitis (n = 9), or epistaxis (n = 8). Unilateral opacification of the maxillary sinus (n = 12) was the most common radiographic finding. Histologically, the tumors exhibited the characteristic features of ameloblastoma, including peripherally palisaded columnar cells with reverse polarity. The majority of the tumors showed a plexiform growth pattern. Fifteen tumors demonstrated surface epithelial derivation. Surgical excision is the treatment of choice, ranging from conservative surgery (polypectomy) to more aggressive surgery (radical maxillectomy). Five patients experienced at least 1 recurrence, usually within 1 year of initial surgery. With follow-up intervals of up to 44 years (mean, 9.5 years), all 24 patients were alive without evidence of disease or had died of unrelated causes, without evidence of disease.
CONCLUSIONS: Primary ameloblastoma of the sinonasal tract is rare. In contrast to their gnathic counterparts, sinonasal tract tumors have a predilection for older age men. Therapy should be directed toward complete surgical resection to prevent local tumor recurrence.
PubMed ID: 9477098
Article Size: 1 MB
 

A rationale for conservative management of microscopic papillary carcinoma of the thyroid gland: a clinicopathologic correlation of 90 cases.

Rassael H, Thompson LD, Heffess CS.
Eur Arch Otorhinolaryngol. 1998;255(9):462-7.
Microscopic papillary carcinoma of the thyroid gland (MPC) measuring < or = 1.0 cm in diameter has a generally benign outcome, but is often overtreated with additional surgery. Ninety cases of MPC and 77 cases of nonmicroscopic papillary carcinoma of the thyroid gland (non-MPC) from 1970 to 1980 were retrieved from the Endocrine Registry of the Armed Forces Institute of Pathology (AFIP), Washington, D.C. Histologic features and patient follow-up were analyzed. Twenty-one patients with MPC had multifocal disease within the affected thyroid lobe, while a further 15 had either bilateral or intraglandular spread. Four of 10 patients who had additional surgery were found to have additional foci of tumor. Fourteen patients with lymph node metastases at initial surgery had no subsequent nodal metastases. All patients were either alive without disease or had died of unrelated causes after an average follow-up period of 17.3 years. Of the 77 non-MPC patients, 13 developed lymph node metastases or local recurrences, and one died of metastatic disease. Sixty-four of these patients were alive without evidence of disease after an average follow-up of 22 years. Present findings show that while MPC may present with perithyroidal lymph node metastases (15.56%), patients do not develop clinical tumors in the remaining thyroid tissue. Our experience indicates that close clinical follow-up without additional surgery is the preferable management for patients with MPC.
PubMed ID: 9833215
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Primary smooth muscle tumors of the thyroid gland.

Thompson LD, Wenig BM, Adair CF, Shmookler BM, Heffess CS.
Cancer. 1997 Feb 1;79(3):579-87.
BACKGROUND: Primary smooth muscle tumors of the thyroid gland are rare. To date, there are few cases reported of primary thyroid leiomyomas and leiomyosarcomas.
METHODS: One leiomyoma and four leiomyosarcomas arising within the thyroid gland were identified in the files of the Endocrine Tumor Registry of the Armed Forces Institute of Pathology. Histologic and immunohistochemical features were reviewed and follow-up obtained.
RESULTS: The patients included 2 females, ages 56 and 64 years, and 3 males, ages 45, 68, and 83 years. The patients presented with a mass in the thyroid gland that had increased in size over a number of months. All the tumors originated within a single lobe of the thyroid gland and measured from 1.1 to 9 cm in greatest dimension. Histologically, there was a fascicular pattern of growth comprised of spindle-shaped cells with blunt-ended nuclei. The leiomyoma was encapsulated, cytologically bland, and amitotic; the leiomyosarcomas were invasive with increased cellularity, pleomorphism, a high mitotic rate, necrosis, and hemorrhage. Immunohistochemical staining showed reactivity with vimentin, smooth muscle actin, muscle specific actin, and desmin. The patient with the leiomyoma was alive without evidence of disease 11 years after the initial presentation, with surgical resection as the only treatment. Three of the patients with leiomyosarcomas were dead within 2 years of diagnosis, in spite of aggressive therapeutic intervention. The remaining patient was still alive 10 months after initial presentation with multiple lung metastases.
CONCLUSIONS: Smooth muscle tumors of the thyroid gland are distinctive tumors. Leiomyosarcomas can be distinguished from anaplastic carcinoma, although patient outcome is uniformly unfavorable.
PubMed ID: 9028371
Article Size: 1 MB
 

Diagnostically challenging lesions in head and neck pathology.

Thompson LD.
Eur Arch Otorhinolaryngol. 1997;254(8):357-66.
There are a variety of diagnostically challenging lesions in the head and neck region. Contact ulcer usually occurs within specific clinical parameters (vocal abuse, post-intubation and gastro-esophageal reflux), which should be documented in correlation with the granulation tissue-like response affecting the posterior vocal cords. Spindle squamous cell carcinoma (carcinosarcoma) presents a variably cellular spindle cell proliferation, often with surface epithelial ulceration. The clinical presentation of a firm, polypoid mass in the larynx, combined with the histomorphologic features of a spindle cell tumor, can be confirmed to be of epithelial origin when a portion of the overlying epithelium is seen to blend with the spindle cell component, or when ancillary studies authenticate the epithelial origin of the tumor. The diagnosis of a verrucous squamous cell carcinoma can only be made accurately with an accurate clinical history. The very well differentiated histologic appearance, a broad pushing border of infiltration, a bland epithelial proliferation with scant mitotic activity and ‘church-spire’-type keratosis coupled with the clinical presentation of a large, locally destructive lesion, can confirm the diagnosis of verrucous carcinoma. A wide variety of disorders can result in midline destructive disease clinically, but a specific etiology must be sought to provide appropriate clinical management. Angiocentric T/NK-cell lymphoma of the sinonasal tract is one such disease. The atypical lymphoid cells are usually angiocentric and angiodestructive in their growth pattern. Identification of the atypical cells in the early stages of disease may be difficult, often requiring multiple biopsies over time with the application of immunohistochemical stains or molecular studies to accurately identify the nature of the infiltrate. Cystic squamous cell carcinoma in the neck is almost always a manifestation of metastatic tumor and not a brachiogenic carcinoma. When specific histomorphologic features are noted (a large, unfilled cyst lined by a ribbon-like or endophytic growth of a ‘transitional’-appearing squamous epithelium with a limited degree of anaplasia), most of these tumors demonstrate primaries in Waldeyer’s ring, often of a very small size. Adequate clinical work-up (pan-endoscopy, extensive radiographic imaging and random biopsies or prophylactic tonsillectomy) is mandatory in order to limit the radiation-therapy ports and to document the location of the primary, yielding an excellent long-term prognosis.
PubMed ID: 9332890
Article Size: <1 MB
 

Oncocytomas of the submandibular gland. A series of 22 cases and a review of the literature.

Thompson LD, Wenig BM, Ellis GL.
Cancer. 1996 Dec 1;78(11):2281-7.
BACKGROUND: Oncocytomas are benign salivary gland neoplasms that represent approximately 1.5% of all salivary gland tumors. Oncocytomas of the submandibular gland, however, are decidedly uncommon.
METHODS: Twenty-two cases of submandibular gland oncocytomas from the files of the Oral and Otolaryngic Tumor Registries of the Armed Forces Institute of Pathology were reviewed, and analysis of the histologic criteria, histochemical and immunohistochemical reactions, and ultrastructural and clinical follow-up data was performed.
RESULTS: The patients included 11 females and 11 males, age 21-88 years, with a mean age at presentation of 58.7 years. Clinically, the tumors were generally asymptomatic masses in the submandibular gland that increased in size over a period ranging from several weeks to 20 years and were occasionally associated with pain (n = 9). The tumors ranged in greatest dimension from 0.7 to 7 cm and were circumscribed to encapsulated. Histologically, the tumors were characterized by large epithelial cells with eosinophilic, granular cytoplasm. The cytoplasm stained positively with stains used to demonstrate mitochondria (phosphotungstic acid-hematoxylin, Novelli, Cresylecht violet V, and Kluver-Barrera Luxol fast blue stains). Immunohistochemical reactions demonstrated an epithelial origin (keratin and epithelial membrane antigen), whereas markers for myoepithelial derivation (S-100 protein, actin, and glial fibrillary acidic protein) were not identified. At the time this study was conducted, all patients with submandibular oncocytomas were either alive without evidence of disease or had died without evidence of recurrent disease, with surgical resection the only treatment.
CONCLUSIONS: Submandibular gland oncocytomas are rare, benign tumors. The tumor cells are filled with mitochondria, which are easily demonstrated by histochemical reactions. Complete surgical resection is adequate therapy.
PubMed ID: 8940996
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Pharyngeal dermoids (‘hairy polyps’) as accessory auricles.

Heffner DK, Thompson LD, Schall DG, Anderson V.
Ann Otol Rhinol Laryngol. 1996 Oct;105(10):819-24.
The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory ‘ears’ (auricles) without the growth potential of a teratoma.
PubMed ID: 8865778
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Lymphoid changes of the nasopharyngeal and palatine tonsils that are indicative of human immunodeficiency virus infection. A clinicopathologic study of 12 cases.

Wenig BM, Thompson LD, Frankel SS, Burke AP, Abbondanzo SL, Sesterhenn I, Heffner DK.
Am J Surg Pathol. 1996 May;20(5):572-87.
We report 12 cases in which the histomorphologic changes of the nasopharyngeal tonsils (adenoids) or palatine tonsils suggest infection with the human immunodeficiency virus (HIV). The patients included 10 men and two women, aged 20 to 42 years (median, 33 years). The clinical presentation included airway obstruction, pharyngitis, fever, and a tonsillar or adenoidal mass lesion. Histologic evaluation of the excised adenoids or tonsils in 10 of the cases demonstrated a spectrum of changes including florid follicular hyperplasia, follicle lysis, attenuated mantle zone, and the presence of multinucleated giant cells (MGC). The latter characteristically localized adjacent to the surface or tonsillar crypt epithelium. Two of the 12 cases showed marked lymphoid depletion with absent germinal centers, plasmacytosis, and stromal vascular proliferation. Immunohistochemical evaluation for HIV p24 core protein showed reactivity in 10 of 12 cases localized to follicular dendritic cell network (FDC), the MGC, scattered interfollicular lymphoid cells, and cells identified within the surface or crypt epithelium. Localization of viral RNA by in situ hybridization paralleled the HIV p24 immunohistochemical findings. Additional significant findings included the presence of both CD-68 and S-100 protein in the MGC and the presence of S-100 protein in dendritic cells. Other than HIV, no microorganisms were identified. At the time of presentation, eight patients were not known to be a risk for HIV infection, nor were they known to be HIV infected or suffering from AIDS. In these patients, HIV infection was suspected on the basis of the histologic changes seen in the resected tonsillar and adenoidal tissue. Serologic evaluation (by enzyme-linked immunosorbent assay), confirmed the presence of HIV infection. Our findings suggest the possibility of HIV dissemination through the upper aero-digestive tract mucosa via target cells, such as intraepithelial dendritic cells, submucosal macrophages, and T-lymphocytes. Subsequent presentation of viral antigens to the tonsillar and adenoidal lymphoid tissues results in enlargement of these structures that clinically may simulate a neoplastic proliferation but causes histomorphologic changes that are highly suspicious for HIV infection even in asymptomatic HIV-positive patients.
PubMed ID: 8619422
Article Size: 4 MB
 

Replication of HIV-1 in dendritic cell-derived syncytia at the mucosal surface of the adenoid.

Frankel SS, Wenig BM, Burke AP, Mannan P, Thompson LD, Abbondanzo SL, Nelson AM, Pope M, Steinman RM.
Science. 1996 Apr 5;272(5258):115-7. — Comment in: Science. 1996 Nov 15;274(5290):1067-8.
Human immunodeficiency virus-type 1 (HIV-1) replicates actively in infected individuals, yet cells with intracellular depots of viral protein are observed only infrequently. Many cells expressing the HIV-1 Gag protein were detected at the surface of the nasopharyngeal tonsil or adenoid. This infected mucosal surface contained T cells and dendritic cells, two cell types that together support HIV-1 replication in culture. The infected cells were multinucleated syncytia and expressed the S100 and p55 dendritic cell markers. Eleven of the 13 specimens analyzed were from donors who did not have symptoms of acquired immunodeficiency syndrome (AIDS). The interaction of dendritic cells and T cells in mucosa may support HIV-1 replication, even in subclinical stages of infection.
PubMed ID: 8600520
Article Size: 2.5 MB
 

Langerhans cell histiocytosis of the thyroid: a series of seven cases and a review of the literature.

Thompson LD, Wenig BM, Adair CF, Smith BC, Heffess CS.
Mod Pathol. 1996 Feb;9(2):145-9.
Langerhans cell histiocytosis is a rare disorder, with a few reports describing isolated thyroid gland involvement. We report seven cases, which included four females and three males ranging in age from 2 months to 55 years, with a median age of 37 years. Histologically, the cases demonstrated either diffuse or focal involvement of the thyroid gland by Langerhans cell histiocytes, characterized by bean-shaped, lobated, folded nuclei. In association with the histiocytic infiltrate, there was a prominent eosinophilic cellular component, as well as destruction of the thyroid follicles. All cases occurred in a background of lymphocytic thyroiditis. One case demonstrated adenomatoid nodules, whereas another had a microscopic papillary carcinoma. Immunohistochemical staining demonstrated positive reactivity with S-100 protein, lysozyme, and KP-1. Four patients with isolated thyroid disease, treated by surgical resection alone, are alive without systemic disease from 3 to 19 years after initial presentation. The three patients with systemic disease died within 1 year of the initial diagnosis with disease-related complications. Localized disease portends a favorable prognosis as compared to the thyroid involvement as part of systemic disease.
PubMed ID: 8657721
Article Size: 3 MB
 

Peripheral Nerve Sheath Tumors of the Thyroid Gland: A Series of Four Cases and a Review of the Literature.

Thompson LD, Wenig BM, Adair CF, Heffess CS.
Endocr Pathol. 1996 Winter;7(4):309-318.
Primary peripheral nerve sheath tumors (PNSTs) of the thyroid gland are exceptionally rare. Two schwannomas and two malignant PNSTs (MPNSTs), arising primarily within the thyroid gland, were identified in the files of the Endocrine Tumor Registry at the Armed Forces Institute of Pathology. The patients included two females, age 69 and 80 yr, and two males, age 18 and 33 yr. The patients presented with a mass in the thyroid gland confined to a single lobe of the thyroid without involvement of the cervical neck region. None of the patients had a history of neurofibromatosis. The benign tumors were encapsulated, one of them cystic, with the characteristic cellular and nuclear features of schwannomas. The MPNSTs were invasive tumors, effacing the thyroid parenchyma, with a fascicular pattern of growth composed of neural appearing cells with increased cellularity, increased mitotic activity, and with focal necrosis. Immunoreactivity for 5100 protein and vimentin was seen in all tumors. The patients with schwannomas, treated only by surgical resection, were alive without evidence of disease, over a period of 5-33 yr. Both patients with MPNSTs died of the disease 8 mo and 42 mo, respectively, with widely disseminated disease. Primary thyroid PNSTs are exceptionally rare tumors. MPNSTs, in this limited experience, have a fatal outcome irrespective of aggressive adjuvant therapy.
PubMed ID: 12114802
Article Size: 5 MB
 

Langerhans cell histiocytosis isolated to the thyroid gland.

Thompson LD.
Eur Arch Otorhinolaryngol. 1996;253(1-2):62-5.
Isolated Langerhans cell histiocytosis of the thyroid is an extremely rare occurrence, with only one case previously reported. A case of Langerhans cell histiocytosis isolated to the thyroid gland associated with lymphocytic thyroiditis is presented and clinical implications are discussed.
PubMed ID: 8932433
Article Size: <1 MB
 

Large-loop excision of the transformation zone: effect on the pathologic interpretation of resection margins.

Montz FJ, Holschneider CH, Thompson LD.
Obstet Gynecol. 1993 Jun;81(6):976-82.
OBJECTIVE: To determine the effect of large-loop excision of the transformation zone (LLETZ) on the ability to interpret adequately the pathologic specimen and surgical margins.
METHODS: Fifty consecutive LLETZ specimens were used for repeat histopathologic assessment with emphasis on the interpretability of the surgical specimen and margin. All reevaluations were performed by a single pathologist. Complete lesion evaluability was defined as satisfactory accuracy of the histologic diagnosis and the ability to evaluate thoroughly all surgical margins. Medical records of the patients from whom the specimens were obtained were reviewed and analyzed for possible correlates to the status of specimen interpretability.
RESULTS: Histologic accuracy was sufficient in 46 cases (92%). Extensive heat distortion precluded full assessment of the ectocervical margins in ten (20%) and the endocervical margins in 22 (44%) of the cases. There was no difference in complete lesion evaluability whether LLETZ was performed solely for treatment in cases suitable for ablative procedures or for both diagnosis and treatment in patients who traditionally would have undergone a cone biopsy. If the latter group (N = 25) was analyzed separately, extensive heat distortion made histopathologic diagnosis impossible in four cases (16%) and precluded full assessment of the ectocervical margin in eight (32%) and the endocervical margin in 12 (48%).
CONCLUSION: The high rate of surgical-margin thermal destruction, with related limitation of interpretability, may represent a serious diagnostic and therapeutic limitation of the LLETZ procedure when considered as an alternative to cold knife conization.
PubMed ID: 8497366
Article Size: 2.5 MB
 

Dedifferentiated chondrosarcomas of the larynx: a report of two cases and review of the literature.

Garcia RE, Gannon FH, Thompson LD.
Laryngoscope. 2002 Jun;112(6):1015-8.
OBJECTIVES: To analyze the long-term clinical outcome of dedifferentiated chondrosarcoma or chondrosarcoma with additional malignant mesenchymal component (CAMMC) of the larynx and compare the results with those of axial chondrosarcomas.
STUDY DESIGN/METHODS: Two patients with CAMMC of the larynx (0.03%) were retrospectively identified within the archives of the Armed Forces Institute of Pathology between 1970 and 2001. We compared the clinical and histologic features of these two cases with those reported in the English literature (Medline 1966-2001) (Table I).
RESULTS: Patient no. 1 was a 67-year-old man who presented with a 12-month history of hoarseness and was found to have a 4-cm mass involving the cricoid cartilage. Enucleation was performed and histologically demonstrated a dedifferentiated chondrosarcoma. Without additional intervention, the patient died after 136 months without evidence of disease. Patient no. 2 was a 41-year-old man who also presented with a 12-month history of hoarseness and dysphagia and was found to have a 5-cm mass involving the cricoid cartilage. A total laryngectomy was performed for the dedifferentiated chondrosarcoma. He is alive without evidence of disease at last contact (91 mo).
CONCLUSION: CAMMC of the larynx are rare tumors but have a better prognosis than their axial counterparts (mean, 6 mo). Initial voice-sparing surgery can be followed with more aggressive surgery if recurrences develop.
PubMed ID: 12160266
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Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases.

Thompson LD.
Am J Surg Pathol. 2002 May;26(5):551-66.
No comprehensive series has evaluated the histologic features of pheochromocytoma to separate benign from malignant pheochromocytoma by histomorphologic parameters only. Fifty histologically malignant and 50 histologically benign pheochromocytomas of the adrenal gland were retrieved from the files of the Armed Forces Institute of Pathology. The patients included 43 females and 57 males, with an age range of 3-81 years (mean 46.7 years). Patients usually experienced hypertension (n = 79 patients). The mean tumor size was 7.2 cm (weight was 222 g). Histologically, the cases of malignant pheochromocytomas of the adrenal gland more frequently demonstrated invasion (vascular [score = 1], capsular [score = 1], periadrenal adipose tissue [score = 2]), large nests or diffuse growth (score = 2), focal or confluent necrosis (score = 2), high cellularity (score = 2), tumor cell spindling (score = 2), cellular monotony (score = 2), increased mitotic figures (>3/10 high power fields; score = 2), atypical mitotic figures (score = 2), profound nuclear pleomorphism (score = 1), and hyperchromasia (score = 1) than the benign tumors. A Pheochromocytoma of the Adrenal gland Scaled Score (PASS) weighted for these specific histologic features can be used to separate tumors with a potential for a biologically aggressive behavior (PASS > or =4) from tumors that behave in a benign fashion (PASS <4). The pathologic features that are incorporated into the PASS correctly identified tumors with a more aggressive biologic behavior. Application of these criteria to a large cohort of cases will help to elucidate the accuracy of this grading system in clinical practice.
PubMed ID: 11979086
Article Size: 3 MB
 

Adenoma versus carcinoid tumor of the middle ear: a study of 48 cases and review of the literature.

Torske KR, Thompson LD.
Mod Pathol. 2002 May;15(5):543-55.
Carcinoid tumors and adenomas of the middle ear are rare neoplasms of indeterminate relationship to one another. Indeed, the literature is devoid of a large comprehensive series that evaluates the clinical, histologic, and immunophenotypic features of these tumors and their potential relationship. Forty-eight cases of middle ear adenoma between 1970 and 1995 were identified in the files of the Armed Forces Institute of Pathology. All cases were evaluated for cytomorphology and architectural pattern, in addition to their reactivity with various immunohistochemical reagents. Clinical follow-up was also obtained. A comprehensive review of the literature was performed with an eye toward correlating any distinct differences or similarities between carcinoid tumors and adenomas of the middle ear. The patients included 21 women and 27 men, aged 20 to 80 years (mean, 45.0 y). Patients experienced hearing loss, mass, and/or pain for a mean duration of 1.7 years. The mean tumor size was 0.8 cm, with six tumors extending beyond the middle ear. Histologically, the tumors were moderately cellular and unencapsulated, arranged in glandular, trabecular, and solid patterns composed of small cells with ‘salt and pepper’ nuclear chromatin distribution. The tumor cells were immunoreactive with keratin, keratin 7, chromogranin, and human pancreatic polypeptide. All patients had surgery. No patients died with their disease (mean follow-up, 15.7 y). Eight patients developed recurrences that were treated surgically and were without evidence of disease at last follow-up (mean, 15.1 y). Our study and the review of the literature showed adenomas and carcinoid tumors of the middle ear to be essentially indistinguishable benign tumors. Middle ear adenoma most correctly describes their morphologic features and clinical behavior, although neuroendocrine adenoma of the middle ear may be a more accurate designation.
PubMed ID: 12011260
Article Size: 3 MB
 

Spindle cell (sarcomatoid) carcinomas of the larynx: a clinicopathologic study of 187 cases.

Thompson LD, Wieneke JA, Miettinen M, Heffner DK.
Am J Surg Pathol. 2002 Feb;26(2):153-70.
Laryngeal spindle cell (sarcomatoid) carcinomas are uncommon tumors, frequently misdiagnosed as reactive lesions or mesenchymal malignancies. The records of 187 patients with tumors diagnosed as laryngeal spindle cell (sarcomatoid) carcinoma were retrieved from the files of the Otorhinolaryngic Tumor Registry of the Armed Forces Institute of Pathology. There were 174 men and 13 women, 35-92 years of age (average, 65.6 years). Nearly all patients experienced hoarseness (n = 165 [88%] patients) for a mean duration of 11.0 months. Patients admitted to smoking (n = 162 [87%] patients) and/or alcohol use (n = 90 [48%] patients). Most tumors were glottic (n = 132 [71%]), T1 (n = 111 [59%]), 1 and polypoid (n = 185 [99%]), with a mean tumor size of 1.8 cm. Histologically, squamous cell carcinoma (n = 157 [84%]) was noted, ulcerated, and blended with the spindle cell component, which was most frequently arranged in a storiform pattern (n = 92 [49%] tumors). Foci of benign or malignant cartilage and/or bone (n = 13 [7%]) were noted in the spindle cell component. All patients were treated with surgery (n = 90 [48%] patients) or surgery with radiation (n = 97 [52%] patients). Recurrences developed in 85 (45%) patients. Overall, T1 glottic tumors managed by complete surgical eradication had the best outcome (mean follow-up, 7.8 years).
PubMed ID: 11812937
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Hodgkin-like transformation of a marginal zone B-cell lymphoma of the larynx.

Fung EK, Neuhauser TS, Thompson LD.
Ann Diagn Pathol. 2002 Feb;6(1):61-6.
Primary larynx lymphomas, specifically of the mucosa-associated lymphoid tissue, are a rare but documented phenomenon. Transformation of any type of lymphoma that has the presence of Reed-Sternberg cells is unusual in lymph nodes and exceptional in extranodal sites. Herein, we report the first case (to the best of our knowledge in a review of the English literature [MEDLINE 1966-2001]) in which both of these unusual findings are present; that is, an extranodal marginal zone B-cell lymphoma of laryngeal mucosa-associated lymphoid tissue with Hodgkin-like transformation. The patient is a 78-year-old man who presented with intermittent shortness of breath, progressive dysphagia, and intermittent hoarseness. On examination, a large mass of the left supraglottic larynx was identified with a ‘ball-valve’ effect into the laryngeal inlet with inspiration. Examination of the neck showed no palpable masses. Histologic examination of the incisional biopsy showed replacement of the submucosa by sheets of atypical monocytoid B cells (CD20+, CD79a+, lambda+, CD3-) characterized by nuclear atypia, mitotic activity, plasmacytoid differentiation, and restricted for lambda light chains. Dutcher bodies were easily identified. Interspersed throughout the neoplastic lymphoid population were numerous Reed-Sternberg cells and variants immunoreactive for CD30 and CD15 and nonreactive for CD45RB. The patient was treated with 44 cGy to the neck and larynx and was alive and free of disease at last contact, 2.6 years after the original presentation.
PubMed ID: 11842381
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Telecytology: intraobserver and interobserver reproducibility in the diagnosis of cervical-vaginal smears.

Alli PM, Ollayos CW, Thompson LD, Kapadia I, Butler DR, Williams BH, Rosenthal DL, O’leary TJ.
Hum Pathol. 2001 Dec;32(12):1318-22.
Telecytologic diagnosis of cervical-vaginal smears is potentially useful because it could allow more efficient use of cytopathologist resources and expertise. A pathologist in one location could, in principle, review cytotechnologists’ findings using a video display hundreds or thousands of miles away. Currently, bandwidth restrictions limit practical implementation of such a system to review of fields that had been selected for review by the cytotechnologist. The purpose of our investigation was to evaluate how well this type of review correlates with a review in which the entire slide is available for examination by the pathologist. We prospectively selected 100 consecutive cervical-vaginal smears over an 11-day period in August 1999. For each smear, 4 to 12 fields containing abnormal cells from each slide were digitally imaged. Each of 3 pathologists reviewed all digitized images and all glass slides. Diagnoses based on selected digitized images were compared with those based on conventional pathologist review. The kappa statistic, a measure of chance-corrected agreement (reproducibility), was calculated in each setting. Overall, intraobserver and interobserver reproducibility of cervical-vaginal smear diagnoses is fair to excellent. The use of remote digital images for pathologist review did not introduce large (2-step) diagnostic disagreements. The disagreement between a pathologist’s glass slide and digital diagnoses is less than that for different pathologists reviewing glass slides, although interobserver differences were even greater in the interpretation of digital images.
PubMed ID: 11774163
Article Size: <1 MB
 

Giant cell tumor of the larynx: a clinicopathologic series of eight cases and a review of the literature.

Wieneke JA, Gannon FH, Heffner DK, Thompson LD.
Mod Pathol. 2001 Dec;14(12):1209-15.
True giant cell tumors of the larynx (GCTL) are quite rare, and only individual case reports are documented in the literature. Eight cases of GCTL were identified in the Otorhinolaryngic Pathology Tumor Registry between 1966 and 2000. There were 2 women and 6 men, ages 26 to 62 years (mean, 44.5 yrs). Patients presented with a palpable neck mass (n = 5), airway obstruction (n = 3), hoarseness (n = 3), and dysphagia (n = 2). All tumors involved the thyroid cartilage, a few with local extension. The mean tumor size was 4.1 cm. Histologically, the tumors showed no connection to the surface epithelium and arose in sites of ossification. The tumors had an expansile, infiltrative growth and consisted of numerous multinucleated osteoclast-like giant cells within a cellular stroma composed of plump, oval mononuclear cells. Of interest was that the nuclei of the giant cells were similar to the nuclei of the stromal cells. Treatment included biopsy only with adjuvant therapy (n = 2), local resection (n = 3), and total laryngectomy (n = 3). Follow-up showed 5 patients were alive without evidence of disease (mean follow-up, 6.9 yrs); 2 died of unrelated causes (mean survival, 22.2 yrs). No patients developed recurrences. GCTL are rare tumors that can cause significant airway obstruction. Complete surgical resection yields an excellent outcome without adjuvant therapy.
PubMed ID: 11743042
Article Size: 2 MB
 

Corticomedullary mixed tumor of the adrenal gland.

Wieneke JA, Thompson LD, Heffess CS.
Ann Diagn Pathol. 2001 Oct;5(5):304-8.
Corticomedullary mixed tumors of the adrenal gland are quite rare, with only five well-documented cases reported in the literature.(1-4) Herein, we report the light microscopic and immunohistochemical features of two cases of this rare tumor. Patient 1 is a 34-year-old woman who presented with hypertension, hair loss, and amenorrhea of 1-year duration. Patient 2 is a 52-year-old woman who presented with flank pain and what appeared to be a renal mass on arteriogram with no history of hypertension, Cushing’s syndrome, or other endocrine abnormalities. At surgery, the tumor was noted to arise from the adrenal gland rather than the kidney and adrenalectomy was performed. In both cases, the surgically resected specimens consisted of a well-circumscribed, single adrenal mass surrounded by a rim of uninvolved adrenal cortical tissue. The tumors were composed of adrenal cortical cells intimately admixed with pheochromocytes. Immunohistochemical studies highlighted these two cellular components. The pheochromocytes were strongly reactive with chromogranin and the sustentacular cells with S-100 protein, whereas the adrenal cortical cells reacted specifically with inhibin. Thus, we report two additional cases of mixed corticomedullary tumor of the adrenal gland. Ann Diagn Pathol 5:304-308, 2001. This is a US government work. There are no restrictions on its use.
PubMed ID: 11598859
Article Size: <1 MB
 

Nodular fasciitis of the external ear region: a clinicopathologic study of 50 cases.

Thompson LD, Fanburg-Smith JC, Wenig BM.
Ann Diagn Pathol. 2001 Aug;5(4):191-8.
Nodular fasciitis (NF), uncommon in the auricular area, is a benign reactive myofibroblastic proliferation that may be mistaken for a neoplastic proliferation. Fifty cases of NF of the auricular region were identified in the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. The patients included 22 females and 28 males, aged 1 to 76 years (mean, 27.4 years). The patients usually presented clinically with a mass lesion (n = 49). Five patients recalled antecedent trauma. The lesions were dermal (n = 28) or subcutaneous (n = 11) in those cases where histologic determination was possible, measuring 1.9 cm on average. The majority of the lesions were circumscribed (n = 38), composed of spindle-shaped to stellate myofibroblasts arranged in a storiform growth pattern, juxtaposed to hypocellular myxoid tissue-culture-like areas with extravasation of erythrocytes. Dense, keloid-like collagen and occasional giant cells were seen (n = 18). Mitotic figures (without atypical forms) were readily identifiable. By immunohistochemical staining, myofibroblasts were reactive with vimentin, actins, and CD68. All patients had surgical excision. Four patients (9.3%) developed local recurrence and were alive and disease free at last follow-up. All patients with follow-up (n = 43) were alive or had died of unrelated causes, without evidence of disease an average 13.4 years after diagnosis. Nodular fasciitis of the auricular area occurs most often in young patients. Because NF is more often dermally situated than extremity NF, it may present with superficial ulceration and/or bleeding. Local recurrence is more frequent because of the difficulty in obtaining complete surgical excision around the ear.
PubMed ID: 11510001
Article Size: <1 MB
 

Mast cell involvement in fibrodysplasia ossificans progressiva.

Gannon FH, Glaser D, Caron R, Thompson LD, Shore EM, Kaplan FS.
Hum Pathol. 2001 Aug;32(8):842-8.
Fibrodysplasia ossificans progressiva (FOP) is a catastrophic genetic disorder of progressive heterotopic ossification associated with dysregulated production of bone morphogenetic protein 4 (BMP4), a potent osteogenic morphogen. Postnatal heterotopic ossification in FOP is often heralded by hectic episodes of severe post-traumatic connective tissue swelling and intramuscular edema, followed by an intense and highly angiogenic fibroproliferative mass. The abrupt appearance, intense size, and rapid intrafascial spread of the edematous preosseous fibroproliferative lesions implicate a dysregulated wound response mechanism and suggest that cells and mediators involved in inflammation and tissue repair may be conscripted in the growth and progression of FOP lesions. The central and coordinate role of inflammatory mast cells and their mediators in tissue edema, wound repair, fibrogenesis, angiogenesis, and tumor invasion prompted us to investigate the potential involvement of mast cells in the pathology of FOP lesions. We show that inflammatory mast cells are present at every stage of the development of FOP lesions and are most pronounced at the highly vascular fibroproliferative stage. Mast cell density at the periphery of FOP lesional tissue is 40- to 150-fold greater than in normal control skeletal muscle or in uninvolved skeletal muscle from FOP patients and 10- to 40-fold greater than in any other inflammatory myopathy examined. These findings document mobilization and activation of inflammatory mast cells in the pathology of FOP lesions and provide a novel and previously unrecognized target for pharmacologic intervention in this extremely disabling disease.
PubMed ID: 11521229
Article Size: 2 MB
 

Laryngeal angiosarcoma: a clinicopathologic study of five cases with a review of the literature.

Loos BM, Wieneke JA, Thompson LD.
Laryngoscope. 2001 Jul;111(7):1197-202.
OBJECTIVE: Primary laryngeal angiosarcoma (LA) is rare without a reported series evaluating these tumors.
STUDY DESIGN/METHODS: Five patients with LA were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology.
RESULTS: Three men and 2 women, aged 29 to 71 years, presented with hoarseness (n = 4) and hemoptysis (n = 1). Two patients had previous neck radiation. The tumors involved the supraglottis (n = 4) with a mean size of 3.1 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, frequent atypical mitotic figures, and hemorrhage. All cases tested (n = 4) demonstrated immunoreactivity with antibodies to Factor VIII-RA and CD34. All patients had surgery followed by postoperative radiation (n = 3 patients). Three patients died with disease (mean, 17 mo), whereas one patient is alive with no evidence of disease at 18 years.
CONCLUSIONS: LA is a rare tumor, frequently associated with previous radiation, usually involving the supraglottis with characteristic histomorphologic and immunophenotypic features. LA has a poor prognosis, making appropriate separation from other conditions important.
PubMed ID: 11568541
Article Size: <1 MB
 

A clinicopathologic and immunohistochemical study of 35 anaplastic carcinomas of the pancreas with a review of the literature.

Paal E, Thompson LD, Frommelt RA, Przygodzki RM, Heffess CS.
Ann Diagn Pathol. 2001 Jun;5(3):129-40.
Anaplastic pancreatic carcinomas are rare tumors, frequently displaying a variety of growth patterns. The literature lacks a comprehensive study of this tumor. Thirty-five cases of anaplastic carcinoma of the pancreas diagnosed between 1955 and 1997 were retrieved from the Endocrine Registry at the Armed Forces Institute of Pathology. Histology, immunophenotype, molecular analysis, and patient follow-up were analyzed. The tumors of 10 women and 25 men, aged 34 to 85 years (mean age at presentation, 62.5 years), were studied. Patients had vague symptoms (weight loss, pain, and fatigue, nausea, or vomiting), lasting an average of 13.2 weeks. The tumors, of an average size of 9.2 cm, were usually in the head or tail of the pancreas. The tumors were widely infiltrative, histomorphologically separated into predominantly large, pleomorphic cell, or spindle cell groups. Tumor phagocytosis and necrosis were noted. Immunohistochemical studies confirmed an epithelial origin with at least one epithelial marker in 78% of the tumors. K-ras mutations by sequence analysis were found in eight of 12 cases tested. Surgical biopsy/excision was used in all patients. Twenty-nine of 35 patients died of disease (average, 5.2 months), three died with no evidence of disease (average, 56.9 months), and three patients were alive at last follow-up (average, 94.0 months), one with residual disease. There was no statistically significant difference in survival between patients with and without a K-ras mutation. Anaplastic carcinoma of the pancreas usually occurs in the head of the pancreas in older men. The epithelial nature of the pleomorphic cells (giant or spindled) can usually be documented. Patients with K-ras mutations have a shorter survival time, even though the overall prognosis for all anaplastic carcinomas is fatal (93% fatality; average survival, 448 days). Ann Diagn Pathol 5: 129-140, 2001. This is a US government work. There are no restriction on its use.
PubMed ID: 11436166
Article Size: <1 MB
 

Adenosquamous carcinoma of the pancreas: a clinicopathologic series of 25 cases.

Kardon DE, Thompson LD, Przygodzki RM, Heffess CS.
Mod Pathol. 2001 May;14(5):443-51.
BACKGROUND: Adenosquamous carcinoma is a rare aggressive subtype of pancreatic adenocarcinoma. We describe the clinical, pathologic, and molecular characteristics of 25 of these lesions, the largest series to date.
METHODS: Twenty-five cases of adenosquamous carcinoma of the pancreas diagnosed between 1961 and 1994 were retrieved from the files of the Endocrine Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed, histochemical, immunohistochemical, and molecular (k-ras) studies were performed, and patient follow-up was obtained.
RESULTS: The patients included 17 men and eight women, aged 28 to 82 years (mean, 65.4 y). The patients usually experienced weight loss (n = 17) or painless jaundice (n = 11), while also presenting with other abdominal symptoms. The tumors affected the head most frequently (n = 17), followed by the tail (n = 9) or body (n = 4). Five cases involved more than one anatomic region of the pancreas. Microscopically, all tumors demonstrated dual differentiation toward adenocarcinoma and squamous cell carcinoma. All cases tested were immunoreactive with keratin (AE1:AE3 and CK1), whereas other keratin markers were variably expressed: CK5/6 (88%), CK7 (68%), Cam5.2 (41%), and CK20(26%). CA-19-9 (84%) and CEA (74%) were positive in the majority of the cases. K-ras oncogene mutations were identified in seven of 13 cases. All patients died from their disease an average of 5.8 months after diagnosis (range, 1 to 33 months).
CONCLUSIONS: Adenosquamous carcinoma of the pancreas represents a distinct clinical and pathologic entity, demonstrating the expected immunoprofile and k-ras oncogene mutation of a ductal origin, with a worse prognosis than ductal adenocarcinoma.
PubMed ID: 11353055
Article Size: 2.5 MB
 

Splenic inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunophenotypic study of 12 cases.

Neuhauser TS, Derringer GA, Thompson LD, Fanburg-Smith JC, Aguilera NS, Andriko J, Chu WS, Abbondanzo SL.
Arch Pathol Lab Med. 2001 Mar;125(3):379-85.
CONTEXT: Inflammatory pseudotumor is an uncommon and enigmatic lesion. The spindle cells found in this tumor have features of myofibroblasts. Because of the indefinite relationship of these lesions with inflammatory fibrosarcoma and their indefinite biologic behavior, inflammatory pseudotumor is currently classified as inflammatory myofibroblastic tumor (IMT). To date, only case reports or small series have been published on these tumors, which are primary in the spleen.
DESIGN: In this study, we describe the clinical, morphologic, and immunophenotypic findings of 12 cases of splenic IMT and examine their relationship to Epstein-Barr virus (EBV).
RESULTS: The patients included 8 women and 3 men, ranging from 19 to 77 years of age (mean, 53 years; median, 60 years). Demographic data were unavailable for 1 patient. Patients generally presented with abdominal pain (n = 5) and fever (n = 4). Associated lesions included renal cell carcinoma (n = 2), colonic adenocarcinoma (n = 1), and cholecystitis (n = 1). All tumors were composed of a bland spindle cell proliferation in association with a variable mixed inflammatory component. There were 2 growth patterns, namely, a cellular spindle cell pattern and a hypocellular fibrous pattern. An immunohistochemical panel confirmed the myofibroblastic nature of the spindle cells. The spindle cells of 2 cases were immunoreactive for EBV latent membrane protein 1, whereas 6 of 10 cases were positive for EBV-encoded RNA using in situ hybridization. Follow-up was available for 8 patients; 6 were alive with no evidence of recurrence and 2 were dead of other causes.
CONCLUSION: Splenic IMTs are uncommon lesions that can be distinguished from other conditions using a combination of clinical, histologic, and immunophenotypic findings. Epstein-Barr virus may play a role in the pathogenesis of splenic IMT, and there may be an association of splenic IMT with concomitant disease or malignancy. Most splenic IMTs have an excellent long-term prognosis.
PubMed ID: 11231487
Article Size: <1 MB
 

Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.

Thompson LD, Heffner DK.
Am J Clin Pathol. 2001 Feb;115(2):243-8.
Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular ‘onion-skin’ fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. Necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.
PubMed ID: 11211613
Article Size: <1 MB
 

Primary osteosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 22 cases with a review of the literature.

Gadwal SR, Gannon FH, Fanburg-Smith JC, Becoskie EM, Thompson LD.
Cancer. 2001 Feb 1;91(3):598-605.
BACKGROUND: Primary osteosarcomas of the head and neck in the pediatric age group, not associated with previous irradiation or a known syndrome, are rare. The literature contains several single cases and small study series; however, to the authors’s knowledge, there has been no comprehensive large study to evaluate the clinicopathologic aspects of these tumors.
METHODS: Twenty-two cases of osteosarcomas of the head and neck in patients 18 years of age or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology (AFIP). No secondary sarcomas (radiation-induced or those arising after chemotherapy) or those associated with known syndromes were included. Clinical, radiographic, and histologic features were reviewed, and patient follow-up was obtained.
RESULTS: The patients included 11 girls and 11 boys, 1-18 years of age (mean, 12.2 yrs). Patient symptoms related to tumor location were painless swelling, loss of teeth, headaches, or a mass lesion, present for an average of 5.9 months. No genetic abnormalities were documented. The tumors most frequently involved the mandible (n = 19), followed by the sphenoid sinus (n = 2) and the maxilla (n = 1). The tumors ranged in size from 1.1-10.0 cm (mean, 4.5 cm). All tumors were invasive and malignant by radiology and/or histology. The tumors were Grade 1 (n = 11), Grade 2 (n = 8), or Grade 3 (n = 3). All cases, except one chondroblastic osteosarcoma, were osteoblastic osteosarcomas. Thirteen patients underwent initial surgical resection with (n = 5) or without (n = 9) additional radiation and/or chemotherapy. The remaining 9 patients had an initial biopsy for diagnosis followed by surgery (n = 4) or surgery and radiation and/or chemotherapy (n = 5). Follow-up was available for 19 patients: 13 were alive at last follow-up with no evidence of disease (mean, 13.1 yrs); 1 was alive with disease (1.3 yrs); 3 had died without evidence of disease (mean, 23.2 yrs); and 2 had died of disease (mean, 7.8 yrs). The 3 patients with high-grade osteosarcoma were alive without disease (mean, 20.0 yrs).
CONCLUSIONS: Primary head and neck osteosarcomas in the pediatric population are typically low- to moderate-grade lesions in the mandible. Despite the invasive nature and high grade of a few of these tumors, there is an excellent overall long-term prognosis for patients in this age group with tumors in these locations.
PubMed ID: 11169944
Article Size: <1 MB
 

A clinicopathologic study of minimally invasive follicular carcinoma of the thyroid gland with a review of the English literature.

Thompson LD, Wieneke JA, Paal E, Frommelt RA, Adair CF, Heffess CS.
Cancer. 2001 Feb 1;91(3):505-24.
BACKGROUND: The criteria for minimally invasive (low grade) follicular carcinoma of the thyroid (MI) remain controversial, often resulting in unnecessary treatment.
METHODS: The records of 130 patients with minimally invasive (MI) follicular thyroid carcinoma were retrieved from the files of the Endocrine Tumor Registry of the Armed Forces Institute of Pathology.
RESULTS: Ninety-five patients were confirmed to have MI based on the authors’ criteria of small-to-medium vessel invasion, capsular invasion of up to full thickness, no parenchymal tumor extension, and no tumor necrosis (patients with oxyphilic tumors were excluded). The remaining 35 patients had tumors that were reclassified as ‘not low grade’ based on large vessel invasion, extension into parenchyma, and tumor necrosis (oxyphilic cases excluded). The MI patients included 67 women and 28 men, ages 20-95 years (average, 42.0 years). Nearly all patients presented with a thyroid mass (n = 90 patients). The mean tumor size was 2.8 cm. Histologic features examined for tumor classification included cellularity, capsule nature, capsular invasion, vascular invasion, extension into parenchyma, cytoplasmic oxyphilia, mitotic activity, and necrosis. All patients were treated with surgical excision. Adjuvant radioactive iodine therapy was performed in 24 patients. Five patients developed recurrent disease: four were alive or had died without evidence of disease after additional treatment (mean, 18.1 years), and one patient died with disease (MI tumor) at 15.1 years. All of the remaining patients were disease free (mean follow-up, 16.5 years).
CONCLUSIONS: There are reproducible histologic criteria to diagnose patients with MI follicular carcinoma. The overall excellent long term prognosis and a good patient outcome suggests that no additional surgery is necessary. Copyright 2001 American Cancer Society.
PubMed ID: 11169933
Article Size: 2 MB
 

Minimally invasive follicular thyroid carcinoma.

Heffess CS, Thompson LD.
Endocr Pathol. 2001 Winter;12(4):417-22.
Infiltration of the capsule, vascular invasion, and/or neoplastic extension into the adjacent parenchyma are regarded as prerequisites for the diagnosis of follicular carcinoma. In modern practice, most of these tumors fall into the category of follicular carcinoma, minimally invasive (FCMI) characterized by evidence of limited capsular or vascular invasion with an excellent long-term prognosis and a good patient outcome. Notwithstanding the wide acceptance of the diagnostic criteria established by the World Health Organization for the classification of follicular carcinomas in particular, they have been difficult to apply and have led to a great deal of confusion. This confusion is compounded when applied to ‘low-grade’ or ‘minimally invasive’ follicular carcinoma because of the poor reproducibility of the classification and the variable results reported in the literature. Our surgical colleagues face a similar lack of a standardized treatment for low-grade follicular carcinomas, which leads to unnecessary surgical treatment. Standardization of histologic criteria is necessary to promote confidence and uniformity in the therapeutic approach of these tumors. We believe that a FCMI is defined as an encapsulated follicular tumor (not papillary), with only small to medium vessel invasion within or immediately adjacent to the tumor capsule and/or up to full-thickness capsular transgression without accompanying extension into the thyroid parenchyma with intervening fibrosis. By using these criteria, patients can be managed with conservative surgical excision to yield an excellent long-term patient outcome.
PubMed ID: 11914475
Article Size: 1 MB
 

Sinonasal mucosal malignant melanoma: report of an unusual case mimicking schwannoma.

Kardon DE, Thompson LD.
Ann Diagn Pathol. 2000 Oct;4(5):303-7.
Primary mucosal melanoma of the sinonasal tract is a rare malignancy that has a more aggressive clinical course than its cutaneous counterpart. The histology of these lesions varies, with differing degrees of melanin production and an epithelioid or spindle-cell growth pattern. Cutaneous melanocytic lesions may differentiate in accordance with their neural crest derivation and express morphology similar to nerve sheath tumors. We believe the following case study reports the first instance of a mucosal melanoma with a Schwannian pattern of growth, arising from the nasal cavity of a 26-year-old man. Extracranial meningiomas of the sinonasal tract are rare tumors. These tumors are frequently misclassified, resulting in inappropriate clinical management. To date, there has been no comprehensive study to evaluate the clinicopathologic aspects of meningioma in these anatomic sites. Thirty cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 were retrieved from the files of the Otorhinolaryngic Registry of the AFIP. Histologic features were reviewed, immunohistochemical studies were performed, patient follow up was obtained, and the results were statistically analyzed. The patients included 15 females and 15 males, aged 13 to 88 years (mean, 47.6 yrs). Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement. Symptoms were present for an average of 31.1 months. The tumors affected the nasal cavity (n = 14), nasopharynx (n = 3), frontal sinus (n = 2), sphenoid sinus (n = 2). or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). The tumors ranged in size from 1.0 to 8.0 cm in greatest dimension (mean, 3.5 cm). Radiographic studies demonstrated a central nervous system connection in six cases. The tumors often eroded the bones of the sinuses (n = 18) and involved the surrounding soft tissues, the orbit, and occasionally the base of the skull. Histologically, the tumors demonstrated features similar to intracranial meningiomas. The majority were of the meningothelial type (n = 23), although there were three atypical meningiomas. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (EMA) and vimentin (all tested). The differential diagnosis includes paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma. Surgical excision was used in all patients. Three patients died with recurrent disease (mean, 1.2 yrs), one was alive with recurrent disease (25.6 years), and the remaining 24 patients were alive or had died of unrelated causes (mean, 13.9 yrs) at the time of last follow up (two patients were lost to follow up). Extracranial sinonasal tract meningiomas are rare tumors which need to be considered in the differential diagnosis of sinonasal tumors. A whorled growth pattern and psammoma bodies, combined with positive EMA and vimentin immunohistochemical reactions, can confirm the diagnosis of meningioma. The overall prognosis is good, without a difference in outcome between benign and atypical meningiomas.
PubMed ID: 11073336
Article Size: <1 MB
 

A clinicopathological study of 15 patients with neuroglial heterotopias and encephaloceles of the middle ear and mastoid region.

Gyure KA, Thompson LD, Morrison AL.
Laryngoscope. 2000 Oct;110(10 Pt 1):1731-5.
OBJECTIVES: Heterotopic masses of neuroglial tissue involving non-midline structures, specifically, the middle ear region, are exceptional. The pathogenesis of these lesions and, in particular, their relation to encephaloceles, is uncertain.
STUDY DESIGN AND METHODS: H&E-stained sections from 15 lesions diagnosed as neuroglial heterotopias or encephaloceles involving the middle ear region were reviewed. Radiographic or operative evidence of a central nervous system (CNS) relation and clinical factors possibly related to pathogenesis were analyzed.
RESULTS: All 15 lesions (from six men and nine women; mean age, 49 y; range, 16-67 y), regardless of their relation to the CNS, were composed of varying proportions of neurons and glia with associated chronic inflammatory cells and reactive gliosis. No significant ependymal or choroid plexus component was present. Operative findings revealed that two lesions had definite CNS connections and two were unrelated to the CNS; this relation could not be determined in the remaining cases. Seven of 10 patients for whom clinical information was available had a history of chronic otitis media or mastoiditis or both; four of these seven patients also had a history of previous trauma or surgery. Three patients, including both patients whose lesions had no demonstrable CNS attachment, had no predisposing factors.
CONCLUSIONS: Most neuroglial heterotopias of the middle ear are probably acquired encephaloceles. These lesions occur in older patients than do their midline counterparts. Determination of the relation of these lesions to adjacent CNS structures must be done radiographically or using operative findings, because histology alone cannot be reliably used to render an accurate diagnosis.
PubMed ID: 11037835
Article Size: 1 MB
 

Tonsillar lymphangiomatous polyps: a clinicopathologic series of 26 cases.

Kardon DE, Wenig BM, Heffner DK, Thompson LD.
Mod Pathol. 2000 Oct;13(10):1128-33.
BACKGROUND: Lymphangiomatous polyps are uncommon benign tumors of the tonsils.
METHODS: Twenty-six cases of lymphangiomatous polyps diagnosed between 1980 and 1999 were retrieved from the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. Hematoxylin and eosin-stained slides were reviewed to characterize the histologic features of these tumors. Immunohistochemical stains were performed on 15 cases. Clinical follow-up data were obtained.
RESULTS: The patients included 13 males and 13 females, ages 3 to 63 years (mean, 25.2 years). Patients experienced dysphagia, sore throat, and the sensation of a mass in the throat. Symptoms were present from a few weeks to years. The tonsillar masses were unilateral in all cases. Clinically, the lesions were frequently mistaken for a neoplasm (n = 18 patients). Grossly, all of the lesions were polypoid and measured 0.5 to 3.8 cm (mean, 1.6 cm). Histologically, the polyps were covered by squamous epithelium showing variable epithelial hyperplasia, dyskeratosis, and lymphocytic epitheliotropism. The masses showed a characteristic submucosal proliferation of small to medium-sized, endothelial-lined, lymph-vascular channels lacking features of malignancy. Collagen, smooth muscle, and adipose tissue were present in the stroma. Intravascular proteinaceous fluid and lymphocytes were noted. Immunohistochemical findings confirmed the endothelial origin of the vascular proliferation and a mixed lymphoid population. The differential diagnosis included fibroepithelial polyp, lymphangioma, juvenile angiofibroma, and squamous papilloma. In all patients with follow-up, complete surgical excision was curative (mean follow-up, 5.4 years; range, 1 mo to 14 years).
CONCLUSIONS: We detail the clinical and pathologic features of tonsillar lymphangiomatous polyps. These tumors are uncommon and may clinically be mistaken for a malignant neoplasm. The characteristic histologic features should allow for its correct diagnosis and differentiation from similar appearing tonsillar lesions.
PubMed ID: 11048808
Article Size: 2.5 MB