Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases.

Adrenal, Endocrine
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.

Ear & Temporal Bone, Head & Neck
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.

Head & Neck, Larynx
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
Article Size: <1 MB
 

Hodgkin-like transformation of a marginal zone B-cell lymphoma of the larynx.

Head & Neck, 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
Article Size: <1 MB
 

Telecytology: intraobserver and interobserver reproducibility in the diagnosis of cervical-vaginal smears.

Cervix, Other Anatomic Sites
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.

Head & Neck, Larynx
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.

Adrenal, Endocrine
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.

Ear & Temporal Bone, Head & Neck
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.

Bone, Other Anatomic Sites
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.

Head & Neck, Larynx
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.

Endocrine, Pancreas
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.

Endocrine, Pancreas
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.

Hematopathology, Other Anatomic Sites
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.

Head & Neck, Sinonasal Tract
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.

Bone, Other Anatomic Sites
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.

Endocrine, Thyroid
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.

Endocrine, Thyroid
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.

Head & Neck, Sinonasal Tract
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.

Ear & Temporal Bone, Head & Neck
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.

Head & Neck, Oropharynx
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
 

Renal cell carcinoma to the pancreas in surgical pathology material.

Endocrine, Pancreas
Thompson LD, Heffess CS.
Cancer. 2000 Sep 1;89(5):1076-88.
BACKGROUND: Clear cell carcinomas of the pancreas are rare and more likely represent metastatic renal cell carcinoma (RCC).
METHODS: Twenty-one cases of metastatic RCC to the pancreas were retrieved from the files of the Endocrine Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed, special stains and immunohistochemical studies were performed, and patient follow-up data were obtained.
RESULTS: The patients included 9 women and 12 men ages 47-76 years (mean, 64.4 years). Patients experienced weight loss, abdominal pain, or a mass lesion. The tumors occurred anywhere within the pancreas. The mean size of the tumors was 4.0 cm. Histologically, the tumors were comprised of clear cells with a rich vascular network. The RCC was diagnosed before (n = 17 patients; ages up to 32.7 years) or after (n = 4 patients; ages up to 13.2 years) the pancreatic metastases were discovered. Surgery was used in all patients. Adjuvant chemotherapy was used in 4 patients. From the date of the diagnosis of pancreatic metastasis, 13 patients were dead with disseminated disease (DD) (mean, 4.5 years), and 8 patients were without disease (mean, 9.0 years). From the date of the diagnosis of primary RCC, 13 patients were DD (mean, 12.7 years), and 8 patients were without disease (mean, 24.7 years).
CONCLUSIONS: Although histochemical and immunohistochemical studies may help in the distinction between patients with primary versus metastatic clear cell tumors of the pancreas, clinical confirmation should be obtained. Surgical resection of the pancreatic metastatic disease is suggested, because the patient may still have a prolonged survival.
PubMed ID: 10964338
Article Size: 1 MB
 

Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases.

Hematopathology, Other Anatomic Sites
Neuhauser TS, Derringer GA, Thompson LD, Fanburg-Smith JC, Miettinen M, Saaristo A, Abbondanzo SL.
Mod Pathol. 2000 Sep;13(9):978-87.
Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.
PubMed ID: 11007038
Article Size: 2 MB
 

Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature.

Head & Neck, Sinonasal Tract
Thompson LD, Gyure KA.
Am J Surg Pathol. 2000 May;24(5):640-50.
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 meningi
PubMed ID: 10800982
Article Size: 2 MB
 
 
 
 

Malignant lymphoma of the thyroid gland: a clinicopathologic study of 108 cases.

Endocrine, Thyroid
Derringer GA, Thompson LD, Frommelt RA, Bijwaard KE, Heffess CS, Abbondanzo SL.
Am J Surg Pathol. 2000 May;24(5):623-39.
We report a retrospective clinicopathologic study of 108 primary thyroid gland lymphomas (PTLs), classified using the REAL and proposed WHO classification schemes. The patients included 79 women and 29 men, with an average age of 64.3 years. All patients presented with a thyroid mass. The PTLs were classified as marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) or MZBL (n = 30), diffuse large B-cell lymphoma (DLBCL) with MZBL (n = 36), DLBCL without MZBL (n = 41), and follicle center lymphoma (FCL; n = 1). Excluding the FCL, features of lymphomas of MALT-type were identified in all groups, despite a follicular architecture in 23% of cases. Lymphocytic thyroiditis (LT) was identified in 94%. Ninety-one percent of patients presented with stage IE or IIE disease, whereas 69% had perithyroidal soft tissue infiltration. All patients were treated with surgical excision followed by adjuvant therapy (76%): chemotherapy (15%), radiation (19%), or a combination of radiation and chemotherapy (42%). Disease-specific survival was 82% at last follow up (mean, 82.8 mos) and 79% at 5 years. Statistically, stages greater than IE, presence of DLBCL, rapid clinical growth, abundant apoptosis, presence of vascular invasion, high mitotic rate, and infiltration of the perithyroidal soft tissue were significantly associated with death with disease. No patients with MZBL or stage IE disease died with disease. In summary, PTLs typically occur in middle- to older-aged individuals as a thyroid mass, with a predilection for females. Despite their histologic heterogeneity and frequent simulation of other lymphoma subtypes, virtually all PTLs are lymphomas of MALT-type arising in the setting of LT. Mixed DLBCL and MZBL are common. Overall, PTLs have a favorable outcome with appropriate therapy, but prognosis depends on both clinical stage and histology. MZBL and stage IE tumors have an excellent prognosis, whereas tumors with a large cell component or DLBCL or stage greater than IE have the greatest potential for a poor outcome.
PubMed ID: 10800981
Article Size: 4 MB
 

Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature.

Bone, Other Anatomic Sites
Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LD.
Cancer. 2000 May 1;88(9):2181-8.
BACKGROUND: Primary chondrosarcoma of the head and neck in the pediatric age group is rare. The literature contains several single cases and small series; however, to the authors’ knowledge, there has been no previous comprehensive larger study to evaluate the clinicopathologic aspects of these tumors.
METHODS: Fourteen cases of chondrosarcoma of the head and neck from patients age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. No secondary sarcomas (radiation-induced or arising in association with Maffucci syndrome or Ollier disease) were included. Clinical, radiographic, and histologic features were reviewed and patient follow-up obtained.
RESULTS: The patients included 6 girls and 8 boys ages 3-18 years (mean, 11.8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, headaches, or a mass lesion) were related to tumor location and were present for an average of 7.2 months. No genetic abnormalities were documented. The tumors most frequently involved the maxillary sinus (n=4), followed by the mandible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasopharynx, orbit, and base of the skull. The tumors ranged in size from 2.0 to 15.0 cm (mean, 3.1 cm). All tumors were invasive and malignant as determined by radiology and/or histology. The tumors were Grade 1 (n=9), Grade 2 (n=1), or Grade 3 (mesenchymal, n=2; dedifferentiated n=2). All patients were treated by surgery, followed by radiation (n=5) and/or chemotherapy (n=2). Follow-up was available for 11 patients; all were alive (at a mean of 14.8 years), with only a single patient demonstrating evidence of residual/ recurrent tumor (at 16.6 years).
CONCLUSIONS: Primary head and neck chondrosarcoma in the pediatric population is typically low grade and occurs in the maxillary sinus or mandible. Despite the invasive and high grade nature of some of these tumors, there is an excellent long term prognosis for patients in this age group with tumors in these locations.
PubMed ID: 10813732
Article Size: <1 MB
 

Amyloidosis of the larynx: a clinicopathologic study of 11 cases.

Head & Neck, Larynx
Thompson LD, Derringer GA, Wenig BM.
Mod Pathol. 2000 May;13(5):528-35.
Laryngeal amyloidosis (LA) is uncommon and poorly understood, with limited long-term clinicopathologic and immunophenotypic studies in the literature. Eleven cases of LA were retrieved from the files of the Otorhinolaryngic-Head & Neck Tumor Registry from 1953 to 1990. The histology, histochemistry, immunohistochemistry, and follow-up were reviewed. All patients (three women and eight men) presented with hoarseness at an average age of 37.8 years. The lesions, polypoid or granular, measured an average of 1.6 cm and involved the true vocal cords only (n = 4), false vocal cord only (n = 1), or were transglottic (n = 6). An acellular, amorphous, eosinophilic material was present in the stroma, often accentuated around vessels and seromucous glands, which reacted positively with Congo red. A sparse lymphoplasmacytic infiltrate was present in all cases that demonstrated light chain restriction by immunohistochemistry in three cases (kappa = 2, lambda = 1). Serum and urine electrophoreses were negative in all patients. Treatment was limited to surgical excision, including a single laryngectomy. Six patients manifested either recurrent and/or multifocal/systemic disease: two patients with light chain restriction were dead with recurrent disease (mean, 11.1 years); two patients were dead with no evidence of disease (mean, 31.7 years); and two patients were alive, one with light chain restriction and recurrent and multifocal disease (41.6 years) and one with no evidence of disease after a single recurrence (43.4 years). The remaining five patients were either alive or had died with no evidence of disease an average of 32.4 years after diagnosis. No patient developed multiple myeloma or an overt B-cell lymphoma. LA is an uncommon indolent lesion that may be associated with multifocal disease (local or systemic). The presence of an associated monoclonal lymphoplasmacytic infiltrate and recurrent/multifocal disease in the respiratory or gastrointestinal tract of a few cases and the lack of development of a systemic plasma cell dyscrasia or overt systemic B-cell malignancy suggest that some LA may be the result of an immunocyte dyscrasia or tumor of mucosa-associated lymphoid tissue.
PubMed ID: 10824924
Article Size: 1.5 MB
 

Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.

Neuro (brain), Other Anatomic Sites
Fanburg-Smith JC, Gyure KA, Michal M, Katz D, Thompson LD.
Ann Diagn Pathol. 2000 Apr;4(2):81-7.
Central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.
PubMed ID: 10760321
Article Size: <1 MB
 

Telecytologic diagnosis of breast fine needle aspiration biopsies. Intraobserver concordance.

Articles, Medical Techniques
Briscoe D, Adair CF, Thompson LD, Tellado MV, Buckner SB, Rosenthal DL, O’Leary TJ.
Acta Cytol. 2000 Mar-Apr;44(2):175-80.
OBJECTIVE: To determine the intraobserver concordance between telecytologic and glass slide diagnosis of breast fine needle aspirates.
STUDY DESIGN: Twenty-five cases, originally received in consultation, were each examined by three cytopathologists. An average of seven compressed digital images per case were presented, together with a brief clinical history, using the http protocol and an internet browser.
RESULTS: Agreement between the telecytologic and glass slide diagnosis ranged from 80% to 96%. Nevertheless, two cases that had been unequivocally diagnosed as malignant based upon video images were considered to be benign by the same pathologist when reviewing the glass slides. Both diagnostic confidence and self-concordance were higher for one pathologist having significant previous video microscopy experience.
CONCLUSION: Although intraobserver concordance between telecytologic and glass slide diagnoses of breast fine needle aspirates is high, refinement of existing criteria for diagnosis of malignancy, taking account of the particular limitations associated with telecytologic diagnosis, may be prudent prior to widespread use of telecytology for fine needle aspiration evaluation.
PubMed ID: 10740603
Article Size: 2 MB
 

Primary thyroid teratomas: a clinicopathologic study of 30 cases.

Endocrine, Thyroid
Thompson LD, Rosai J, Heffess CS.
Cancer. 2000 Mar 1;88(5):1149-58.
BACKGROUND: Primary thyroid teratomas are rare thyroid gland neoplasms of germ cell derivation that display features of trilineage differentiation.
METHODS: The histologic and immunophenotypic features of 30 cases of thyroid teratomas were reviewed, patient follow-up was obtained, and the results were analyzed statistically.
RESULTS: The patients included 15 females and 15 males ages newborn-56 years (mean, 12.4 years). All patients presented clinically with a mass in the thyroid, ranging in size from 2.0-13 cm in greatest dimension (mean, 6.0 cm). Histologically, the tumors usually were well circumscribed, although occasionally infiltrative into the thyroid parenchyma. Derivatives of all three germ layers (ectoderm, mesoderm, and endoderm) were present in varying degrees of maturity. The tumors were divided into benign (n = 7 tumors), immature (n = 14 tumors), and malignant (n = 9 tumors) as determined by an increasing percentage of tumor volume comprised of primitive mesenchymal or neural-type tissue. All the microscopically malignant tumors occurred in the adult population. Surgical excision was performed in 28 patients, followed by adjuvant therapy in 5 patients. Follow-up was obtained in 26 patients; 8 patients had died from or with tumor (5 neonates with immature histology and 3 adults with malignant histology; mean, 0.6 years) and 18 patients were alive with no evidence of disease at a mean of 16.9 years of follow-up.
CONCLUSIONS: Thyroid teratomas are rare neoplasms that can be divided into three types depending on the presence and proportion of the immature component. The outcome is dependent largely on the age of the patient, the size of the tumor at the time of initial presentation, and the presence and proportion of immaturity. Surgical excision is the treatment of choice, with adjuvant therapy reserved for the malignant cases. Copyright 2000 American Cancer Society.
PubMed ID: 10699906
Article Size: <1 MB
 

A clinicopathologic series of 22 cases of tonsillar granulomas.

Head & Neck, Oropharynx
Kardon DE, Thompson LD.
Laryngoscope. 2000 Mar;110(3 Pt 1):476-81.
OBJECTIVES: Tonsils are uncommonly affected by granulomatous inflammation, often with an obscure cause. This study attempts to elucidate the nature of tonsillar granulomatous inflammation.
DESIGN: Retrospective clinicopathologic review.
METHODS: Twenty-two cases of tonsillar granulomas diagnosed between 1940 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. H&E slides and a series of histochemical stains were reviewed, and patient follow-up was obtained.
RESULTS: There were 11 males and 11 females, aged 7 to 64 years (mean, 29.9 y). Most of the cases presented bilaterally (n = 19) with sore throat, dysphagia, and/or nasal obstruction. The clinical differential included chronic tonsillitis, tuberculosis, nonspecific infection, sarcoidosis, and a neoplasm. Histologically, the granulomas were focal and scattered, or diffuse, identified in the interfollicular zones (n = 16) and/or the germinal centers (n = 13), and occasionally associated with necrosis (n = 6). Based on histochemical and clinical follow-up information, the etiology of the granulomas included sarcoidosis (n = 8), tuberculosis (n = 3), Hodgkin’s lymphoma (n = 2), toxoplasmosis (n = 1), squamous cell carcinoma (n = 1), and no specific known cause (n = 7). Twelve patients were either alive at last follow-up or had died with no evidence of disease (mean, 12.4 y), and 9 were either alive at last follow-up or had died with disease (mean, 24.9 y). One patient was alive with unknown disease status (lost to follow-up after 13.3 y).
CONCLUSIONS: Although a cause for tonsillar granulomas is frequently identified, a number may not develop an identifiable etiology, with the granulomas probably representing an exaggerated immune response to chronic tonsillitis. However, a careful work-up must be conducted to exclude specific causes and avoid clinical mismanagement.
PubMed ID: 10718441
Article Size: 1 MB
 

Kaposi sarcoma of major salivary gland origin: A clinicopathologic series of six cases.

Head & Neck, Salivary Gland
Castle JT, Thompson LD.
Cancer. 2000 Jan 1;88(1):15-23.
BACKGROUND: Kaposi sarcoma (KS), one of the defining tumors of acquired immune deficiency syndrome (AIDS), is rarely identified in the major salivary glands. To the authors’ knowledge, no previous published series has evaluated the clinicopathologic aspects of this tumor in major salivary glands.
METHODS: Six cases of salivary gland KS, diagnosed between 1970 and 1998, were retrieved from the files of the Oral and Maxillofacial Pathology Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed and special stains, immunohistochemical studies, and in situ hybridization were performed (n = 4). Patient follow-up data were obtained.
RESULTS: The patients included 6 men ages 20-73 years (average, 53.0 years). Patients presented clinically with a mass in the submandibular (n = 4) or parotid (n = 2) gland region. Symptoms were present for a mean of 13.7 months. The tumors measured 1-4 cm (average, 2.5 cm) in greatest dimension. Histologically, the tumors exhibited the usual features of KS: a spindle cell vascular proliferation arranged in fasciculated bundles, variable nuclear pleomorphism, mitotic figures, extravasated erythrocytes, and hyaline globules. Five patients were serologically positive for human immunodeficiency virus (HIV) (three homosexual males, one infected by a contaminated blood transfusion, and one with an unknown risk factor). Human herpesvirus-8 (HHV-8) was present in all cases tested (n = 4). Patients were treated with surgical excision (n = 6), followed by chemotherapy (n = 1) for the single patient with other foci of KS (rectal). Three patients died of AIDS-related infectious complications and one of congestive heart failure, whereas the remaining patients are alive with AIDS but free of salivary gland KS.
CONCLUSIONS: Salivary gland enlargement is frequently identified in HIV positive or AIDS patients. Although rare, it is important to consider KS in the differential diagnosis of other AIDS-related salivary gland manifestations (infections and tumors). Copyright 2000 American Cancer Society.
PubMed ID: 10618601
Article Size: 1 MB
 

Polymorphous low grade adenocarcinoma: a clinicopathologic study of 164 cases.

Head & Neck, Salivary Gland
Castle JT, Thompson LD, Frommelt RA, Wenig BM, Kessler HP.
Cancer. 1999 Jul 15;86(2):207-19.
BACKGROUND: Polymorphous low grade adenocarcinomas (PLGA) are minor salivary gland neoplasms with a predilection for intraoral sites.
METHODS: One hundred sixty-four cases of PLGA diagnosed between 1970-1994 were retrieved from the files of the Armed Forces Institute of Pathology, Washington, DC. Histologic features were reviewed, immunohistochemical studies and prognostic markers were performed, and patient follow-up was obtained. The data were analyzed statistically.
RESULTS: The patients included 109 women and 55 men, ages 23-94 years (average, 57.6 years). The patients usually presented clinically with a palatal mass that ranged in size from 0.4-6 cm (average, 2.2 cm). The tumors were infiltrative and characterized by a polymorphous growth pattern, with individual tumors demonstrating multiple patterns, including solid, ductotubular, cribriform, trabecular, and single file growth. Neurotropism was identified frequently. The neoplastic cells were isomorphic with vesicular nuclei. Mitotic activity was inconspicuous. At an average of 115.4 months after presentation, approximately 97.6% of all patients were either alive or had died without evidence of recurrent disease after treatment with surgical excision only. Four patients had evidence of disease at last follow-up; three had died with evidence of tumor, and one patient was alive with tumor.
CONCLUSIONS: PLGA is a neoplasm of minor salivary gland origin that must be separated from adenoid cystic carcinoma and benign mixed tumor for therapeutic and prognostic considerations. Conservative but complete surgical excision is the treatment of choice for these slow-growing tumors with a low proliferation index; adjuvant therapy does not appear to alter the prognosis.
PubMed ID: 10421256
Article Size: 2 MB
 

A clinicopathologic and immunohistochemical study of 22 intraductal papillary mucinous neoplasms of the pancreas, with a review of the literature.

Endocrine, Pancreas
Paal E, Thompson LD, Przygodzki RM, Bratthauer GL, Heffess CS.
Mod Pathol. 1999 May;12(5):518-28.
Intraductal papillary-mucinous neoplasms (IPMNs) of the pancreas are rare lesions. We undertook this study to analyze these tumors by focusing on the diagnostic criteria and correlating the histologic features with clinical prognosis. Twenty-two cases of IPMN were retrieved from the Endocrine Tumor Registry of the Armed Forces Institute of Pathology. Blocks or unstained slides were available for histochemical and immunohistochemical studies (including proliferative markers and cell cycle regulators) and K-ras oncogene mutations in 15 cases. Patient follow-up was obtained in all of the cases. IPMN occurs in both genders with a slight male predominance, with a mean age at presentation of 64.4 years (range, 48-85 yr). The patients presented with abdominal pain. The neoplasms were radiologically and grossly cystic, usually (18 cases of 22) located in the head of the pancreas. Histologically, the tumors consisted of intraductal papillary proliferations protruding into and expanding the pancreatic ducts. Invasion into the surrounding pancreatic parenchyma was detected in 15 cases. Chronic pancreatitis was present in all of the cases. p27 immunoreactivity always exceeded the immunoreactivity of cyclin E. K-ras oncogene mutations were detected in two cases. Patients were treated with a complete surgical resection (n = 7) or a Whipple procedure (n = 13). Only 2 of 22 patients died of disease (3 died immediately postoperatively and 3 died of unrelated causes), whereas the remaining 14 patients were alive at last follow-up, without evidence of disease, an average of 58.2 months after initial presentation. IPMNs are rare, distinctive neoplasms, with complex intraductal papillae, that can be easily separated from in situ ductal adenocarcinoma and mucinous cystic neoplasms. The high ratio of p27 protein to cyclin E supports the excellent prognosis of these neoplasms, despite the presence of invasion and K-ras oncogene mutation.
PubMed ID: 10349991
Article Size: 8.5 MB
 

Exophytic and papillary squamous cell carcinomas of the larynx: A clinicopathologic series of 104 cases.

Head & Neck, Larynx
Thompson LD, Wenig BM, Heffner DK, Gnepp DR.
Otolaryngol Head Neck Surg. 1999 May;120(5):718-24.
Exophytic and papillary squamous cell carcinomas (SCCs) are uncommon variants of SCC of the upper aerodigestive tract mucosa. The histomorphologic distinction between these variants has not been previously attempted or correlated with prognostic outcome. One hundred four cases of exophytic and papillary SCCs of the larynx were identified in the files of the Armed Forces Institute of Pathology from 1971 to 1991. The patients included 25 women and 79 men, aged 27 to 89 years (average 60.7 years). Patients had hoarseness at presentation, and many patients were using tobacco (n = 87) and/or alcohol (n = 49). Tumors measured up to 6 cm in greatest dimension. The larger tumors were associated with vocal cord impairment (n = 39). Histologically, the SCCs were divided into 2 growth patterns: papillary-frond (n = 12) or broad-based, exophytic (n = 92). Patients were treated with excisional biopsy, vocal cord stripping, and/or laryngectomy, in conjunction with radiation therapy (n = 70). Eighty-seven patients had no evidence of disease at last follow-up (average follow-up 8.6 years). Seventeen patients with an exophytic pattern died with disease (10 disseminated disease; 7 local disease). No patients with papillary patterns died of disease, although there had been 4 recurrences. In conclusion, patients with papillary and exophytic SCCs have a better prognosis than those with conventional SCCs, and the prognosis for those with papillary SCCs is even better.
PubMed ID: 10229599
Article Size: <1 MB
 

Carcinoma Ex-Schneiderian Papilloma (Malignant Transformation): A Clinicopathologic and Immunophenotypic Study of 20 Cases Combined with a Comprehensive Review of the Literature.

Head & Neck, Sinonasal Tract
Nudell J, Chiosea S, Thompson LD.
Head Neck Pathol. 2014 Sep;8(3):269-286.
Schneiderian papilloma (SP) are uncommon tumors with malignant transformation even less common. The histologic criteria to define malignant transformation are not well developed nor is the immunohistochemical profile reported in a large series of carcinomas. 20 cases of malignant transformation of SP included 7 females and 13 males, aged 38-86 years (mean 60.7 years). Patients presented most frequently with a mass (n = 11) and obstructive symptoms (n = 7), present for 38.7 months (mean). Most patients had no previous history of SP (n = 13); metachronous carcinoma was identified in 7 patients an average of 34.4 months after the first diagnosis of SP, with 1-4 recurrences of SP. With a mean size of 4.1 cm, the majority of tumors involved a combination of more than one anatomic site (n = 10), followed by the maxillary sinus only (n = 5) or nasal cavity only (n = 3). Histologically, 17 were inverted and 3 exophytic type SP. There were 17 squamous cell carcinomas, 2 mucoepidermoid carcinomas and 1 sinonasal undifferentiated carcinoma, comprising from 10 to 95 % of the tumor volume. Malignant histologic features included atypical mitoses, necrosis, bone invasion, lymphovascular invasion, decreased transmigrating neutrophils, paradoxical maturation, dyskeratosis and/or perineural invasion (n = 3). Patients tended to present with advanced stage (n = 14, Stage III and IV). Immunohistochemical studies showed positive reactions in the malignancies for CK5/6 (86 %), p63 (86 %), CK7 (luminal, 50 %), p53 (83 %), and p16 (25 %). In situ hybridization detected human papillomavirus in 26 %. Surgery was often accompanied by radiation therapy (n = 13), with a mean of 2.4 years of follow-up. Five patients developed a recurrence between 0.8 and 3.3 years. Carcinomas ex-SP are less common and are associated with better outcome than previously reported. Patients tend to present with a synchronous carcinoma, developing in an inverted type SP, with squamous cell carcinoma the most common malignancy. Development of metachronous carcinomas ex-SP was always preceded by SP recurrence in this series.
PubMed ID: 24519376
Article Size: 1.7 MB
 

Sinonasal tumors: a clinicopathologic update of selected tumors.

Head & Neck, Sinonasal Tract
Slootweg PJ, Ferlito A, Cardesa A, Thompson LD, Hunt JL, Strojan P, Takes RP, Triantafyllou A, Woolgar JA, Rinaldo A, Devaney KO, Barnes L.
Eur Arch Otorhinolaryngol. 2013 Jan;270(1):5-20.
The sinonasal cavities show a wide variety of neoplasms of epithelial, mesenchymal, neural/neuroectodermal or hematopoietic origin. The differential diagnosis for these tumors may be difficult due to overlapping morphologies, variable patterns in ancillary studies, and potentially confusing terminology. In this report, an updated review of the spectrum of neoplasia is provided, using the World Health Organization 2005 classification as a guide. Classic tumors that are generally limited to the sinonasal tract are described and new information regarding molecular pathogenesis is reviewed. Also new entities that have the sinonasal tract as a site of predilection, such as sinonasal renal cell-like adenocarcinoma and NUT midline carcinoma are highlighted.
PubMed ID: 22610012
Article Size: 2.2 MB
 

Intracranial mucocele formation in the context of longstanding chronic rhinosinusitis: A clinicopathologic series and literature review.

Head & Neck, Sinonasal Tract
Lee JT, Brunworth J, Garg R, Shibuya T, Keschner DB, Vanefsky M, Lin T, Choi S, Stea R3 Thompson LD.
Allergy Rhinol (Providence). 2013 Fall;4(3):e166-75.
Chronic rhinosinusitis (CRS) can lead to serious long-term adverse sequelae, particularly if left untreated. The aim of this study was to describe a series of intracranial mucoceles (ICMs) that arose in the context of longstanding CRS combined with a review of the pertinent literature. A retrospective chart review was performed on all patients who developed ICMs in association with CRS between 2003 and 2012. The clinical presentation, radiographic features, surgical approach, intraoperative findings, and patient outcome were examined in the context of a literature review. Sixty-five cases of mucoceles were identified in patients with a history of CRS, of which seven (10.8%) were intracranial. Five patients were men and two were women with a mean age of 42.1 years. Headache, facial pressure, retro-orbital pain, and visual disturbances were the most common presenting symptoms. Five of the seven had previously undergone sinonasal surgery. Imaging studies showed ICMs involving the anterior cranial fossa, two of which were bilateral. Latency between onset of CRS and ICM detection ranged from 3 to 19 years (mean, 9.4 years). All patients underwent endoscopic transnasal drainage with three also requiring a concurrent, open neurosurgical procedure to access the intracranial component. There were no postoperative complications, and no recurrences were observed after a mean follow-up of 2.7 years. ICMs presenting as delayed complications of CRS are uncommon and constitute a surgical challenge. Open, external skull base approaches used in conjunction with transnasal endoscopic drainage procedures may be necessary to achieve successful management of this rare condition.
PubMed ID: 24498523
Article Size: 2.5 MB
 

Double reporting and second opinion in head and neck pathology.

Articles, Medical Techniques
Woolgar JA, Triantafyllou A, Thompson LD, Hunt JL, Lewis JS Jr, Williams MD, Cardesa A, Rinaldo A, Barnes L, Slootweg PJ, Devaney KO, Gnepp DR, Westra WH, Ferlito A.
Eur Arch Otorhinolaryngol. 2014 May;271(5):847-54.
FIRST PARAGRAPH: This editorial aims to discuss the practice of ‘‘double reporting’’ and ‘‘second opinion’’ diagnosis in routine diagnostic pathology interpretation. It does not encompass reviews performed as part of audit and quality assurance functions, but is from the perspective of experienced head and neck and oral and maxillofacial specialists.
PubMed ID: 24435586
Article Size: <1 MB
 

Acute Necrotizing Herpetic Tonsillitis: A Report of Two Cases

Head & Neck, Oropharynx
Borhan WM, Dababo MA, Thompson LD, Saleem M, Pashley N.
Head Neck Pathol. 2015 Mar;9(1):119-22.
The finding of herpetic tonsillitis is rare. Tonsillectomies are usually done for children with recurrent chronic tonsillitis, while viral throat infections are generally self-limiting. We present two cases: A 5 yearold girl, with atypical hemolytic anemia managed with Eculizumab, who presented with a pharyngeal infection and tonsillar enlargement that did not respond to intravenous antibiotics or antifungal therapies; and a 30 yearold man who presented with upper airway obstruction and fever; bilateral tonsillectomies were performed. Histopathological examination showed a necrotizing tonsillitis with numerous ground-glass intranuclear inclusions, characteristic of herpes viral infection, further confirmed by Herpes simplex virus in situ hybridization. Both patients were managed by intravenous Acyclovir, with dramatic improvement.
PubMed ID: 24338612
Article Size: <1 MB
 

Larynx: nodules and polyps.

Head & Neck, Larynx
Thompson LD.
Ear Nose Throat J. 2013 Sep;92(9):426-8.
FIRST PARAGRAPHS: Nodules usually affect the anterior to middle thirds of the true vocal folds, and they are nearly always bilateral. About 1.5% of the overall population suffers from hoarseness; among the most common causes of hoarseness are vocal fold nodules and polyps. Vocal fold nodules and polyps represent reactive changes of the laryngeal mucosa and adjacent stroma that result in benign nodular or polypoid growths. Their etiology is multifactorial, but vocal misuse is one of the most common causes, followed by infection, smoking, and hypothyroidism. Extroverts are more likely to develop nodules and polyps.
PubMed ID: 24057901
Article Size: <1 MB
 

The sinonasal tract: Another potential ‘hot spot’ for carcinomas with transcriptionally-active human papillomavirus.

Head & Neck, Sinonasal Tract
Lewis JS Jr, Westra WH, Thompson LD, Barnes L, Cardesa A, Hunt JL, Williams MD, Slootweg PJ, Triantafyllou A, Woolgar JA, Devaney KO, Rinaldo A, Ferlito A.
Head Neck Pathol. 2014 Sep;8(3):241-249.
While high risk human papillomavirus (HPV) is well established as causative and clinically important for squamous cell carcinoma (SCC) of the oropharynx, its role in non-oropharyngeal head and neck SCC is much less clearly elucidated. In the sinonasal region, in particular, although it is a relatively uncommon site for SCC, as many as 20 % of SCC harbor transcriptionally-active high risk HPV. These tumors almost always have a nonkeratinizing morphology and may have a better prognosis. In addition, specific variants of SCC as well as other rare carcinoma types, when arising in the sinonasal tract, can harbor transcriptionally-active HPV. This article reviews the current literature on HPV in sinonasal carcinomas, attempts to more clearly demonstrate what tumors have it and how this relates to possible precursor lesions like inverted papilloma, and discusses the possible clinical ramifications of the presence of the virus.
PubMed ID: 24338611
Article Size: <1 MB
 

Definitive treatment of androgen receptor–positive salivary duct carcinoma with androgen deprivation therapy and external beam radiotherapy.

Head & Neck, Salivary Gland
Soper MS, Iganej S, Thompson LD.
Head Neck. 2014 Jan;36(1):E4-7.
BACKGROUND: Salivary duct carcinoma (SDC) is an aggressive malignancy with high recurrence rates. Standard management includes surgical resection followed by adjuvant radiation. Androgen receptor positivity has been described to be present in 40% to 90% of SDCs, and a recent case series showed a benefit to androgen deprivation therapy (ADT) in recurrent or metastatic disease.
METHODS AND RESULTS: We present the case of an 87-year-old woman with a locally advanced androgen receptor-positive parotid SDC treated definitively with ADT and external beam radiotherapy, a regimen modeled after the treatment of prostate cancer. She had a complete response on positron emission tomography (PET)/CT scan and had no evidence of disease 24 months after the completion of treatment.
CONCLUSION: To our knowledge, this case report is the first to describe the use of ADT plus radiation to definitively treat SDC. This regimen could be considered in patients with androgen receptor-positive SDCs who are considered unresectable or who refuse surgery.
PubMed ID: 23720164
Article Size: <1 MB
 

Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases.

Head & Neck, Sinonasal Tract
Thompson LD, Penner C, Ho NJ, Foss RD, Miettinen M, Wieneke JA, Moskaluk CA, Stelow EB.
Head Neck Pathol. 2014 Mar;8(1):88-109.
Primary sinonasal tract and nasopharyngeal adenoid cystic carcinomas (STACC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. Eighty-six cases of STACC included 45 females and 41 males, aged 12-91 years (mean 54.4 years). Patients presented most frequently with obstructive symptoms (n = 54), followed by epistaxis (n = 23), auditory symptoms (n = 12), nerve symptoms (n = 11), nasal discharge (n = 11), and/or visual symptoms (n = 10), present for a mean of 18.2 months. The tumors involved the nasal cavity alone (n = 25), nasopharynx alone (n = 13), maxillary sinus alone (n = 4), or a combination of the nasal cavity and paranasal sinuses (n = 44), with a mean size of 3.7 cm. Patients presented equally between low and high stage disease: stage I and II (n = 42) or stage III and IV (n = 44) disease. Histologically, the tumors were invasive (bone: n = 66; neural: n = 47; lymphovascular: n = 33), composed of a variety of growth patterns, including cribriform (n = 33), tubular (n = 16), and solid (n = 9), although frequently a combination of these patterns was seen within a single tumor. Pleomorphism was mild with an intermediate N:C ratio in cells containing hyperchromatic nuclei. Reduplicated basement membrane and glycosaminoglycan material was commonly seen. Necrosis (n = 16) and atypical mitotic figures (n = 11) were infrequently present. Pleomorphic adenoma was present in 9 cases; de-differentiation was seen in two patients. Immunohistochemical studies showed positive reactions for pan-cytokeratin, CK7, CK5/6, CAM5.2, and EMA, with myoepithelial reactivity with SMA, p63, calponin, S100 protein and SMMHC. CD117, CEA, GFAP and p16 were variably present. CK20 and HR HPV were negative. STACC needs to be considered in the differential diagnosis of most sinonasal malignancies, particularly poorly differentiated carcinoma, olfactory neuroblastoma and pleomorphic adenoma. Surgery (n = 82), often accompanied by radiation therapy (n = 36), was generally employed. A majority of patients developed a recurrence (n = 52) 2-144 months after initial presentation. Overall mean follow-up was 19.4 years (range 0.4-37.5 years): 46 patients died with disease (mean 6.4 years); 5 were alive with disease (mean 5.4 years), and 35 patients were either alive or had died of unrelated causes (mean 16.3 years). ACC of the SNT is uncommon. Recurrences are common. The following parameters, when present, suggest an increased incidence of either recurrence or dying with disease: mixed site of involvement, high stage disease (stage IV), skull base involvement, tumor recurrence, a solid histology, perineural invasion, bone invasion, and lymphovascular invasion.
PubMed ID: 24037641
Article Size: 1.5 MB