Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy.

Neuro (brain), Other Anatomic Sites
Rushing EJ, Thompson LD, Mena H.
Ann Diagn Pathol. 2003 Aug;7(4):240-4.
We describe a case of anaplastic astrocytoma in a 14-year-old boy arising at the site of a dysembryoplastic neuroepithelial tumor (DNT) 3 years after combined radiation and chemotherapy. The subtotally excised superficial right temporoparietal tumor was originally diagnosed as mixed oligoastrocytoma in 1974; the patient was treated with radiation therapy postoperatively. One year later he underwent a craniotomy to remove cyst fluid and no change was reported in the size of the residual tumor. Postoperatively, he received a 6-week course of chemotherapy (lovustine, CCNU). He remained clinically and radiographically stable until 3 years later, when seizure activity returned and imaging studies were consistent with tumor recurrence. He was lost to follow-up until 1986, when records showed that he had died. Review of the initial biopsy showed cortical fragments containing abundant calcifications and multinodular structures typical of the complex form of DNT, in addition to specific glioneuronal elements. The Ki-67 labeling index ranged from 0.1% to 3% focally. The specimen from the third surgery showed an anaplastic astrocytoma (Ki-67 up to 12%) and morphologic features characteristic of radiation effect. This is the first documented case of malignant transformation of DNT following radiation and adjuvant chemotherapy. The implications of malignant transformation in subtotally excised complex DNTs and the intriguing issue of the contribution of radiation/chemotherapy are discussed.
PubMed ID: 12913847
Article Size: <1 MB
 

Molluscum contagiosum.

Other Anatomic Sites, Skin
Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Aug;82(8):560.
FIRST PARAGRAPH: Molluscum contagiosum is a virus-induced epithelial hyperplasia produced by a DNA poxvirus. This common disease can be found on the skin and mucosal surfaces. It is acquired by direct contact with an infected individual or, less commonly, by contact with a fomite (e.g., clothing, towels, and toys); autoinoculation is common. Molluscum contagiosum is usually seen in children and young adults; immunocompromised persons are especially vulnerable to infection. Lesions predominantly arise on the skin of the face, neck, eyelids, trunk, and genitalia, as well as on the mucous membranes of these areas (as applicable). The incubation period averages between 2 and 7 weeks, although it can be much longer.
PubMed ID: 14503090
Article Size: <1 MB

Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients.

Adrenal, Endocrine
Wieneke JA, Thompson LD, Heffess CS.
Am J Surg Pathol. 2003 Jul;27(7):867-81.
Adrenal cortical neoplasms in pediatric patients (<20 years) are rare. The clinical manifestations and biologic behavior of these lesions can be quite distinct from their histologically similar counterparts in the adult population, making pathologic criteria for distinguishing benign from malignant tumors equivocal. We undertook a study of 83 adrenal cortical neoplasms to determine if adult clinical and histologic features can be applied to pediatric patients in an outcome-based analysis. Most of the patients (50 girls and 33 boys) presented with hormone-related symptoms present for a mean of 6.8 months. The tumors ranged in size from 2 to 20 cm (mean 8.8 cm). Histologic parameters examined included capsular and/or vascular invasion, extraadrenal soft tissue extension, growth pattern, cellularity, necrosis, cytoplasmic eosinophilia, nuclear pleomorphism, nuclear-to-cytoplasmic ratio, prominent nucleoli, mitotic figures, atypical mitotic figures, bands of fibrosis, and calcifications. Immunophenotypically, there was reactivity with inhibin, vimentin, CK5, and focally with p53 and Ki-67. All patients underwent adrenalectomy, and 20 patients received adjuvant therapy. All patients with tumors classified as adenomas (n = 9) were alive, without evidence of disease (mean 14.7 years), whereas 21 patients with carcinomas had died with disease (mean 2.4 years). Only 31% of histologically malignant tumors behaved in a clinically malignant fashion. Features associated with an increased probability of a malignant clinical behavior included tumor weight (>400 g), tumor size (>10.5 cm), vena cava invasion, capsular and/or vascular invasion, extension into periadrenal soft tissue, confluent necrosis, severe nuclear atypia, >15 mitotic figures/20 high power fields, and the presence of atypical mitotic figures. Vena cava invasion, necrosis, and increased mitotic activity (>15 mitotic figures/20 high power fields) independently suggest malignant clinical behavior in multivariate analysis.
PubMed ID: 12826878
Article Size: 2 MB
 

Intratracheal ectopic thyroid tissue: a case report and literature review.

Endocrine, Thyroid
Byrd MC, Thompson LD, Wieneke JA.
Ear Nose Throat J. 2003 Jul;82(7):514-8.
We discuss a case of intratracheal ectopic thyroid tissue (ETT) that was retrieved from the files of the Otorhinolaryngic–Head and Neck Pathology Registry at the Armed Forces Institute of Pathology. The patient was a 54-year-old man who had a history of papillary thyroid carcinoma, which had been treated with a subtotal thyroidectomy. During routine follow-up 4 years later, the patient’s primary care physician detected an elevated thyroglobulin level. Further referrals and evaluations revealed that the patient had intratracheal ETT. The patient refused to undergo surgical excision and remains without evidence of recurrent carcinoma. In a MEDLINE literature review, we found only 13 other well-documented cases of intratracheal ETT since 1966; in all but two cases, patients had benign disease. Once the possibility of thyroid carcinoma has been eliminated by histologic examination, intratracheal ETT can be managed by complete surgical excision with the prospect of an excellent long-term clinical outcome.
PubMed ID: 12955837
Article Size: <1 MB

Necrotizing sialometaplasia.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Penner CR, Thompson LD.
Ear Nose Throat J. 2003 Jul;82(7):493-4.
FIRST PARAGRAPH: Necrotizing sialometaplasia is a benign, self-limited, reactive inflammatory process that involves salivary glands. It occurs in middle-aged patients; men are affected slightly more often than women. Patients may experience an antecedent event (e.g., surgery, radiation, or trauma) up to 3 weeks before necrotizing sialometaplasia appears clinically, but many are asymptomatic. A submucosal nodular swelling will give way to an ulcerative, crater-like lesion that usually measures less than 3 cm in its greatest dimension.
PubMed ID: 12955830
Article Size: <1 MB

Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation.

Head & Neck, Sinonasal Tract
Thompson LD, Miettinen M, Wenig BM.
Am J Surg Pathol. 2003 Jun;27(6):737-49.
Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n = 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.
PubMed ID: 12766577
Article Size: 2 MB
 
 
 
 

Polymorphous hemangioendothelioma of the neck.

Head & Neck, Neck
Tadros M, Rizk SS, Opher E, Thompson LD.
Ann Diagn Pathol. 2003 Jun;7(3):165-8.
Polymorphous hemangioendotheliomas are rare, low-grade borderline malignant vascular tumors of endothelial cell origin. To the best of our knowledge (MEDLINE 1966-2002), there have been nine cases of polymorphous hemangioendothelioma reported in the English literature. Most of the initial patients reported were men, but we present the third case in a woman. Her previous radiation history to the neck makes this report unique. Polymorphous hemangioendothelioma is characterized by the variety of patterns of growth within and between tumors, making histologic recognition of the tumor difficult. Because management remains conservative via wide local excision, the misdiagnosis of this lesion as a malignancy has possible treatment implications. Alternatively, the high propensity for local recurrence underscores the necessity for accurate classification of the neoplasm and close clinical follow-up.
PubMed ID: 12808568
Article Size: <1 MB
 

Tracheopathia osteoplastica.

Head & Neck, Larynx
Penner CR, Thompson LD.
Ear Nose Throat J. 2003 Jun;82(6):427.
FIRST PARAGRAPH: Tracheopathia osteoplastica (tracheobronchopathia osteochondroplastica) is a segmental degenerative disorder of the tracheobronchial tree. It is characterized by multiple submucosal cartilaginous and osseous nodules of various sizes that cause a narrowing of the upper respiratory tract. This disorder is most common in elderly men, and it is occasionally associated with chronic inflammation or with trauma. Tracheopathia osteoplastica can manifest clinically as nonspecific signs and symptoms, although stridor and dyspnea are common. Radiologic studies may suggest the diagnosis if scalloped nodular calcified opacities are seen in the submucosa. The diagnosis is confirmed after endoscopic and pathologic examination.
PubMed ID: 12861866
Article Size: <1 MB

Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system.

Head & Neck, Sinonasal Tract
Thompson LD, Wieneke JA, Miettinen M.
Am J Surg Pathol. 2003 May;27(5):594-611.
Primary sinonasal tract mucosal malignant melanomas are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. A total of 115 cases of sinonasal tract mucosal malignant melanoma included 59 females and 56 males, 13-93 years of age (mean 64.3 years). Patients presented most frequently with epistaxis (n = 52), mass (n = 42), and/or nasal obstruction (n = 34) present for a mean of 8.2 months. The majority of tumors involved the nasal cavity (n = 34), septum alone, or a combination of the nasal cavity and sinuses (n = 39) with a mean size of 2.4 cm. Histologically, the tumors were composed of a variety of cell types (epithelioid, spindled, undifferentiated), frequently arranged in a peritheliomatous distribution (n = 39). Immunohistochemical studies confirmed the diagnosis of sinonasal tract mucosal malignant melanomas with positive reactions for S-100 protein, tyrosinase, HMB-45, melan A, and microphthalmia transcription factor. Sinonasal tract mucosal malignant melanomas need to be considered in the differential diagnosis of most sinonasal malignancies, particularly carcinoma, lymphoma, sarcoma, and olfactory neuroblastoma. Surgery accompanied by radiation and/or chemotherapy was generally used. The majority of patients developed a recurrence (n = 79), with 75 patients dying with disseminated disease (mean 2.3 years), whereas 40 patients are either alive or had died of unrelated causes (mean 13.9 years). A TNM-type classification separated by anatomic site of involvement and metastatic disease is proposed to predict biologic behavior.
PubMed ID: 12717245
Article Size: 2 MB
 

Primary parathyroid hyperplasia.

Endocrine, Parathyroid
Penner CR, Thompson LD.
Ear Nose Throat J. 2003 May;82(5):363.
FIRST PARAGRAPH: Parathyroid hyperplasia is classified as either primary, secondary, or tertiary. Primary parathyroid hyperplasia occurs in approximately 15% of patients with hyperparathyroidism. Most cases are sporadic, and they usually occur in patients who are middle-aged and older. Approximately 20% of all cases of primary chief-cell hyperplasia are associated with one of the multiple endocrine neoplasia syndromes. Symptoms are referable to the level and duration of serum calcium elevation, although routine biochemical testing has led to an increase in the identification of asymptomatic patients. Biochemically, ionized serum calcium levels are elevated, and serum phosphorus concentrations are lowered. Technetium-99m sestamibi imaging successfully localizes as many as 60% of hyperplastic glands, although this technique is significantly more effective in localizing adenomas and carcinomas. Therefore, at least two glands should be examined histologically to confirm the diagnosis.
PubMed ID: 12789760
Article Size: <1 MB

Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature.

Head & Neck, Sinonasal Tract
Knott PD, Gannon FH, Thompson LD.
Laryngoscope. 2003 May;113(5):783-90.
OBJECTIVES/HYPOTHESIS: Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors.
STUDY DESIGN: Retrospective review. METHODS: Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic-Head and Neck Registry of the Armed Forces Institute of Pathology.
RESULTS: Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up.
CONCLUSIONS: Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
PubMed ID: 12792311
Article Size: 2 MB
 

Malignant giant cell tumor of the sphenoid.

Head & Neck, Sinonasal Tract
Chan J, Gannon FH, Thompson LD.
Ann Diagn Pathol. 2003 Apr;7(2):100-5.
Malignant giant cell tumors (MGCTs) of the sphenoid sinus are extremely rare neoplasms. They are challenging to diagnose and difficult to treat because of their skull base location. To the best of our knowledge, we report the first case of a primary MGCT of the sphenoid arising in a patient with Paget’s disease. A 77-year-old man presented with epistaxis and a history of Paget’s disease. There was normal cranial nerve function although radiographic images disclosed a large mass centered in the sphenoid sinus and extending into the ethmoid and maxillary sinuses. Excisional biopsy revealed a MGCT composed of a cellular stroma with increased mitotic activity and necrosis with giant cells present throughout. Additional therapy was declined and the patient died with disease 7 months later. Because of their rarity, no treatment guidelines exist for the management of MGCTs of the sphenoid. We discuss both the diagnostic and therapeutic considerations based on a review of the pertinent literature.
PubMed ID: 12715335
Article Size: <1 MB
 

Fibrous dysplasia of bone.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Apr;82(4):259.
FIRST PARAGRAPH: Fibrous dysplasia (fibro-osseous metaplasia) is one of a diverse group of diseases that are characterized by alterations in bone growth. It is a developmental, tumor-like process of unknown etiology. Its initial clinical sign is usually a painless enlargement of the affected bone. It occurs in equal proportions in males and females, most often during the first two decades of life.
PubMed ID: 12735156
Article Size: <1 MB

Tonsil with Tangier disease.

Head & Neck, Oropharynx
Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Mar;82(3):178.
FIRST PARAGRAPH: Tangier disease is a rare autosomal-recessive inherited disorder that is caused by a defect in chromosome 9q31. It is characterized by a severe deficiency or absence of high-density lipoproteins in plasma. A defect in cellular cholesterol removal results in the massive, abnormal accumulation of cholesterol esters in macrophages in many tissues. Although this accumulation is most conspicuous in the tonsils, progressive accretion of these esters also occurs in nerves (neuropathy) and vessels (atherosclerosis). The pathognomonic finding is a low plasma cholesterol concentration accompanied by normal or elevated triglyceride levels and large, lobulated, hyperplastic, bright orange-yellow tonsils and adenoid tissue. Affected families have been identified on Tangier Island, Va., in Chesapeake Bay as well as in Missouri, Kentucky, and Europe.
PubMed ID: 12696235
Article Size: <1 MB

Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature.

Ear & Temporal Bone, Head & Neck
Thompson LD, Bouffard JP, Sandberg GD, Mena H.
Mod Pathol. 2003 Mar;16(3):236-45.
‘Primary’ ear and temporal bone meningiomas are tumors that are frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. To date, a large clinicopathologic study of meningiomas in this anatomic site has not been reported. Thirty-six cases of ear and temporal bone meningiomas diagnosed between 1970 and 1996 were retrieved from our files. Histologic features were reviewed, immunohistochemical analysis was performed (n = 19), and patient follow-up was obtained (n = 35). The patients included 24 females and 12 males, aged 10-80 years (mean, 49.6 years), with female patients presenting at an older age (mean, 52.0 years) than male patients (mean, 44.8 years). Patients presented clinically with hearing changes (n = 20), otitis (n = 7), pain (n = 5), and/or dizziness/vertigo (n = 3). Symptoms were present for an average of 24.6 months. The tumors affected the middle ear (n = 25), external auditory canal (n = 4), or a combination of temporal bone and middle ear (n = 7). The tumors ranged in size from 0.5 to 4.5 cm in greatest dimension (mean, 1.2 cm). Radiographic studies demonstrated a central nervous system connection in 2 patients. Histologically, the tumors demonstrated features similar to those of intracranial meningiomas, including meningothelial (n = 33), psammomatous (n = 2), and atypical (n = 1). An associated cholesteatoma was identified in 9 cases. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (79%) and vimentin (100%). The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and middle ear adenoma. Surgical excision was used in all patients. Ten patients developed a recurrence from 5 months to 2 years later. Five patients died with recurrent disease (mean, 3.5 years), and the remaining 30 patients were alive (n = 25, mean: 19.0 years) or had died (n = 5, mean: 9.5 years) of unrelated causes without evidence of disease. We conclude that extracranial ear and temporal bone meningiomas are rare tumors histologically similar to their intracranial counterparts. They behave as slow-growing neoplasms with a good overall prognosis (raw 5-y survival, 83%). Extent of surgical excision is probably the most important factor in determining outlook because recurrences develop in 28% of cases.
PubMed ID: 12640104
Article Size: 2 MB
 

Cystic chondromalacia of the ear.

Ear & Temporal Bone, Head & Neck
Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Feb;82(2):104-5.
FIRST PARAGRAPH: Idiopathic cystic chondromalacia (endochondral pseudocyst of the auricle) is a benign cystic degenerative lesion of the auricular cartilage. The lesion appears as a painless unilateral swelling along the upper half of the ear, usually in the area of the scaphoid or triangular fossae adjacent to the helix. The disorder affects young and middle-aged men more often than it does women. Trauma is an associated, although not proven, etiologic factor.
PubMed ID: 12619465
Article Size: <1 MB

Ameloblastoma.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Thompson LD.
Ear Nose Throat J. 2003 Jan;82(1):19.
FIRST PARAGRAPH: Ameloblastomas are locally aggressive gnathic tumors that have a high propensity for recurrence. They are believed to arise from remnants of the odontogenic epithelium or the developing enamel organ. They occur in patients over a wide spectrum of ages and equally among the sexes, as a slow-growing, often asymptomatic, locally invasive tumor. Radiographic images usually demonstrate a multilocular, expansile radiolucency of bone, usually of the posterior mandible.
PubMed ID: 12610896
Article Size: <1 MB

Causes of death of patients with laryngeal cancer.

Head & Neck, Larynx
Ferlito A, Haigentz M Jr, Bradley PJ, Suárez C, Strojan P, Wolf GT, Olsen KD, Mendenhall WM, Mondin V, Rodrigo JP, Boedeker CC, Hamoir M, Hartl DM, Hunt JL, Devaney KO, Thompson LD, Rinaldo A, Takes RP.
Eur Arch Otorhinolaryngol. 2014 Mar;271(3):425-34.
Despite remarkable advances in the care of patients with laryngeal cancer over the past several decades, including a growing awareness of therapeutic complications and attention to quality of life, little is known about the causes of mortality in this population. In addition to the laryngeal malignancy itself, acute and late or chronic treatment-associated causes, second primary cancers, intercurrent disease and psychosocial factors are all responsible for patient morbidity and mortality. We examine the current literature related to the causes of death in patients with laryngeal cancer, in the hope of guiding future interventions to improve the longevity and quality of life of individuals with this cancer.
PubMed ID: 23591796
Article Size: <1 MB
 

Otic polyp.

Ear & Temporal Bone, Head & Neck
Thompson LD.
Ear Nose Throat J. 2012 Nov;91(11):474-5.
FIRST PARAGRAPHS: An uncommon entity, otic polyp arises in response to a long-standing inflammatory or infectious process of the middle ear, most often in young boys. An otic, or aural, polyp is a benign proliferation of chronic inflammatory cells and granulation tissue that is usually lined with benign reactive epithelium. These lesions arise in response to a long-standing inflammatory or infectious process of the middle ear. Otic polyps are uncommon; when they do occur, they usually affect young patients. They are more common in males than in females.
PubMed ID: 23288791
Article Size: <1 MB

Osteosarcoma.

Head & Neck, Oral Cavity/Gnathic (Jaw)
Thompson LD.
Ear Nose Throat J. 2013 Jul;92(7):288-90.
FIRST PARAGRAPHS: Osteosarcoma affects the mandible and the maxilla differently, with mandibular tumors tending to arise from the body of the mandible while maxillary tumors arise from the alveolar ridge and sinus. Osteosarcoma is a mesenchymal malignancy in which the neoplastic cells synthesize and secrete the organic components of bone matrix. While it is the most common primary tumor of bone, it is very uncommon overall.
PubMed ID: 23904301
Article Size: <1 MB

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

Head & Neck, Sinonasal Tract
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
Article Size: MB
 

Invited Review: An update on salivary gland pathology.

Head & Neck, Salivary Gland
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
Article Size: <1 MB
 

Endolymphatic sac tumor.

Ear & Temporal Bone, Head & Neck
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
Article Size: <1 MB

Mucocele: Retention and extravasation types.

Head & Neck, Salivary Gland
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
Article Size: <1 MB

Wegener granulomatosis.

Head & Neck, Sinonasal Tract
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
Article Size: <1 MB

Nodular fasciitis.

Head & Neck, Neck
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
Article Size: <1 MB

Metastatic renal cell carcinoma to the thyroid gland: a clinicopathologic study of 36 cases.

Endocrine, Thyroid
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
Article Size: 2 MB
 

Hemangioma of the parotid.

Head & Neck, Salivary Gland
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
Article Size: <1 MB

Relapsing polychondritis.

Ear & Temporal Bone, Head & Neck
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
Article Size: <1 MB

Subacute (de Quervain’s) thyroiditis.

Endocrine, Thyroid
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
Article Size: <1 MB

Rhinoscleroma.

Head & Neck, Sinonasal Tract
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
Article Size: <1 MB
 

Chondrosarcoma of the larynx: a clinicopathologic study of 111 cases with a review of the literature.

Head & Neck, Larynx
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
Article Size: 1 MB
 

Basaloid squamous cell carcinoma of the sinonasal tract.

Head & Neck, 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.

Endocrine, Pancreas
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.

Endocrine, Pancreas
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
Article Size: <1 MB
 

The clinical importance of cystic squamous cell carcinomas in the neck: a study of 136 cases.

Head & Neck, Oropharynx
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.

Endocrine, Thyroid
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.

Head & Neck, Sinonasal Tract
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.

Endocrine, Thyroid
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
Article Size: <1 MB
 

Primary smooth muscle tumors of the thyroid gland.

Endocrine, Thyroid
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.

Head & Neck, Larynx
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.

Head & Neck, Salivary Gland
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
Article Size: <1 MB
 

Pharyngeal dermoids (‘hairy polyps’) as accessory auricles.

Head & Neck, Oropharynx
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
Article Size: <1 MB
 

Lymphoid changes of the nasopharyngeal and palatine tonsils that are indicative of human immunodeficiency virus infection. A clinicopathologic study of 12 cases.

Head & Neck, Oropharynx
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.

Head & Neck, Oropharynx
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.

Endocrine, Thyroid
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.

Endocrine, Thyroid
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.

Endocrine, Thyroid
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.

Cervix, Other Anatomic Sites
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.

Head & Neck, Larynx
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
Article Size: <1 MB