Variants of squamous cell carcinoma of the upper aerodigestive tract.
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Carcinoma, Squamous Cell↗
Biomedical subjects
B M Wenig
Publications and source records attributed to B M Wenig.
Primary smooth muscle tumors of the thyroid gland.
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.
Aged↗
Pigmented extraadrenal paragangliomas. A clinicopathologic and immunohistochemical study of five cases.
BACKGROUND: Pigmented extraadrenal paragangliomas are unusual neoplasms that have rarely been reported in the literature. METHODS: The clinical, pathologic, and immunohistochemical features of five cases of pigmented extraadrenal paragangliomas were reviewed. RESULTS: The patients were 2 women and 3 men within the ages of 17 and 56 years (mean age: 36.5). Two neoplasms were located in the lumbar spine, one in the urinary bladder, one in the anterior mediastinum, and one in the retroperitoneum. Clinically, one patient with spinal paraganglioma presented with symptoms of numbness and weakness of the lower extremities whereas the second patient had low back pain of several weeks' duration. The paraganglioma of the bladder occurred in a pregnant woman who had symptoms of dysuria and microscopic hematuria whereas the patient with an anterior mediastinal tumor presented with chest pain. No clinical history was obtained from the patient with the retroperitoneal tumor. None of the patients had a history of hypertension. Grossly, the tumors were described as well-circumscribed, soft, and slightly hemorrhagic, and measured from 2 to 9 cm in greatest dimension. Histologically, the five tumors displayed characteristics similar to those described in these tumors, mainly the presence of an organoid or zellballen growth pattern. In addition, they contained moderate amounts of intracellular melanin pigment that focally obscured the true nature of the lesion. Immunohistochemically, four cases were positive for chromogranin whereas S-100 protein was detected in the sustentacular cells in four cases. Follow-up information ranging from 6 months to 18 years for 3 patients revealed that the patients were alive and well without recurrence or metastasis. One patient with spinal paraganglioma was lost to follow-up, and the patient with mediastinal paraganglioma was a recent case and therefore the behavior of the paraganglioma could not be assessed. CONCLUSIONS: The current study expands the morphologic spectrum of extraadrenal paragangliomas and emphasizes the need to consider these tumors in the differential diagnosis of pigmented neoplasms. These findings suggest that the presence of melanin pigment does not alter the behavior of these neoplasms.
Adolescent↗
Laryngeal paragangliomas. A review and report of a single case.
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Humans↗
Pancreatic mucinous cystic neoplasm with sarcomatous stroma: a report of three cases.
Three patients with pancreatic mucinous cystic neoplasms having a sarcomatous stroma are reported. The tumors occurred in two women and one man, aged 48, 66, and 67 years, respectively. Symptoms included epigastric pain or a palpable abdominal mass or both. Radiographically, all the tumors were large, multicystic, and located in the tail of the pancreas. Histologically, the cystic component was lined by mucin-producing, columnar to cuboidal epithelium composed of benign to atypical to overtly malignant cells (cystadenocarcinoma). Immunohistochemistry showed the epithelial component of all three tumors to be positive with cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, and the pancreatic epithelial mucin antibodies CA 19-9 and DUPAN 2. In all three cases, an undifferentiated malignant spindle cell (sarcomatous) stroma was intimately associated with the epithelial component. The sarcomatous component showed variable reactivity with vimentin (all three cases), muscle-specific and smooth-muscle actin (all three cases), S-100 protein (1/3), Leu-7 (one case), and estrogen and progesterone receptors (in two cases). Two patients died with widespread abdominal disease within 15 months of diagnosis, one of whom had an omental metastasis entirely composed of the sarcomatous stroma. The third patient was alive and free of disease at 16 months after diagnosis. Our findings document the existence of a sarcomatous component in pancreatic mucinous cystic neoplasms; this component appears to be responsible for the highly malignant behavior of the tumors.
Aged↗
Cytokeratin 20 immunoreactivity distinguishes Merkel cell (primary cutaneous neuroendocrine) carcinomas and salivary gland small cell carcinomas from small cell carcinomas of various sites.
Cytokeratin 20 (CK20) is a low-molecular-weight cytokeratin (CK) that shows restricted expression in the gastrointestinal epithelium, urothelium, and Merkel cell. Recent studies have suggested that since Merkel cell (primary cutaneous neuroendocrine) carcinomas are consistently CK20-positive, this feature may help to distinguish it from pulmonary small cell carcinomas. However, only limited numbers of these tumors have been studied, and the pattern of CK20 expression in other small cell carcinomas has not been established. Therefore, we studied CK20 expression in small cell carcinomas from a wide variety of sites. Immunohistochemical study was performed on paraffin sections using CK20 antibody, coupled with antigen retrieval by pressure cooking in citrate buffer. The cases included 34 Merkel cell carcinomas and 89 small cell carcinomas from various sites (pulmonary, 37; gastrointestinal tract, nine; pharynx and tongue, two; sinonasal tract, three; salivary gland, five; larynx, nine; breast, two; thymus, three; uterine cervix and corpus, 12, prostate, three; urinary bladder, two; kidney, one; pancreas, one). In addition, all cases were immunostained with pan-CK (MNF-116) and low-molecular-weight CK (CAM5.2) antibodies to ascertain their epithelial nature. With the exception of one case, all Merkel cell carcinomas were CK20-positive; and 30 of the 33 cases showed a punctate pattern. Almost 100% of tumor cells were positive, except for two cases that showed staining of 10% and 30% of tumor cells, respectively. Among the other small cell carcinomas, only five cases were CK20-positive, including one of 37 pulmonary (40% cells positive in punctate pattern), one of 11 cervical (10% cells positive), and three of five salivary gland (100% cells positive). We conclude that CK20-positivity in a small cell carcinoma of uncertain origin strongly predicts a diagnosis of Merkel cell carcinoma, especially if the majority of tumor cells are positive. A negative CK20 reaction can practically rule out Merkel cell carcinoma, provided that an effective antigen retrieval technique is used and appropriate staining is obtained with other cytokeratin antibodies. The frequent CK20 positivity observed in salivary gland small cell carcinomas in this series suggests that at least some of them may be more closely related biologically to Merkel cell carcinoma than to pulmonary-type small cell carcinoma. This may explain why they are far less clinically aggressive than other small cell carcinomas.
Biomarkers, Tumor↗
Clinicopathological consultation. Ear cholesteatoma versus cholesterol granuloma.
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Cholesteatoma, Middle Ear↗
Human immunodeficiency virus-1 infection of the lymphoid tissues of Waldeyer's ring.
Human immunodeficiency virus-1 (HIV-1) infection is a fatal retroviral infection that may first present clinically as enlargement of the lymphoid tissues of Waldeyer's ring. These tissues are a major site of viral replication. The presence of the virus in these tissues causes a unique constellation of diagnostic histopathologic features, including florid follicular hyperplasia, follicle lysis, and productively HIV-1-infected multinucleated giant cells of probable dendritic cell origin. Serologic evaluation is confirmatory of HIV infection. With the recent advances in antiretroviral chemotherapy, the early institution of which may significantly prolong life and disease-free interval, the recognition of the clinical and pathologic parameters of HIV-related enlargement of Waldeyer's ring tissues is essential.
Disease-Free Survival↗
Microsporidium-associated sinusitis.
Two cases of biopsy-proven Microsporidium-associated chronic sinusitis in HIV-seropositive patients are presented. Spores of Septata intestinalis were identified by light microscopy and confirmed by electron microscopy in each case. Both patients displayed severe deficiencies of nasal mucosa CD4-positive cells, demonstrated by immunohistochemical methods. Only two other cases of Septata intestinalis-associated sinusitis have been reported previously. Our observations agree with the theory that functional defects in local mucosal immunity may partially explain the acquisition of opportunistic mucosal infections in many HIV-seropositive patients.
AIDS-Related Opportunistic Infections↗
Active replication of HIV-1 at the lymphoepithelial surface of the tonsil.
Cells that are infected with HIV-1 were visualized at the mucosal surface of the nasopharyngeal and palatine tonsils in 14 specimens from patients with CD4+ T-cell counts of 200 to 900/microliter and 2- to 10-year histories of HIV-1 infection. Most of the cells with intracellular HIV-1 protein were small but multinucleated. The majority of these syncytia could be double labeled for HIV-1 RNA and a dendritic cell marker S100. In the palatine tonsil, the infected cells were not found in the stratified squamous epithelium that is adjacent to the pharynx. Instead, the S100+ infected syncytia were localized to the surface of tonsil invaginations or crypts. This mucosa, termed lymphoepithelium, contains antigen-transporting M cells that lie above regions where S100+ dendritic cells are juxtaposed with CD4+ lymphocytes. Likewise, infected cells were found in lymphoepithelium and not respiratory epithelium of nasopharyngeal tonsils or adenoids. We propose that lymphoepithelia, the histological term that describes the specialized regions where antigens access mucosa-associated lymphoid tissue, are sites where HIV-1 replication can be enhanced in syncytia derived from dendritic cells.
Adenoids↗
Oncocytomas of the submandibular gland. A series of 22 cases and a review of the literature.
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.
Adenoma, Oxyphilic↗
Replication of HIV-1 in dendritic cell-derived syncytia at the mucosal surface of the adenoid.
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.
Adenoids↗
Hyperthyroidism caused by an ectopic TSH-secreting pituitary tumor.
A woman developed what appeared to be typical Graves' disease in 1965 at the age of 45 years. After 9 years of antithyroid drug therapy, she was treated with radioiodine. Ten years later (1985) she developed postablative hypothyroidism. Despite replacement doses of thyroxine that resulted in thyroid hormone levels that were in the hyperthyroid range, TSH levels remained elevated. Initial biochemical studies, including a high alpha-subunit to TSH ratio, suggested a pituitary TSH-secreting tumor, but a CT scan of the sella turcica was normal. In 1994, while undergoing an otolaryngologic examination, the patient was found to have a nasopharyngeal mass lesion, which was ultimately shown histologically and immunohistochemically to be an ectopic pituitary tumor. Resection of the mass restored TSH and alpha-subunit levels to normal. This patient probably represents the first ectopic TSH-secreting pituitary tumor to be reported.
Adenoma, Pleomorphic↗
Lymphoid changes of the nasopharyngeal and palatine tonsils that are indicative of human immunodeficiency virus infection. A clinicopathologic study of 12 cases.
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.
Adenoids↗
Schneiderian-type mucosal papillomas of the middle ear and mastoid.
Five cases of schneiderian-type mucosal papillomas arising in the middle ear space are reported. The patients were all women, ranging in age from 19 to 57 years (median, 31 years). Clinical complaints--unilateral conductive hearing loss, pain, or otorrhea--ranged from those lasting several months to recurrent problems spanning 20 years. All of the patients had a history of chronic otitis media predating the development of the papillomas; none of the patients had a history of sinonasal or nasopharyngeal schneiderian-type papillomas. Clinically, three patients had intact tympanic membranes, while the other two patients had perforated tympanic membranes through which a bulging polypoid mass was identified. Radiographic studies showed opacification of the middle ear space without evidence of osseous destruction. The intraoperative findings were of polypoid lesions filling the middle ear space, including involvement of the eustachian tube orifice. Histologically, the tumors were identical to sinonasal schneiderian papillomas. Immunohistochemical evaluation for human papillomavirus was negative. Surgical excision is the treatment of choice. In four of the patients, recurrent tumor was identified, necessitating additional surgery. In only one patient did the initial surgery result in complete ablation of the tumor. All patients are alive and free of recurrent disease over periods ranging from 6 months to 120 months (median, 84 months).
Adult↗
Botryomycosis ('bacterial ball') of the sinonasal tract caused by Pseudomonas aeruginosa.
BACKGROUND: Botryomycosis is a chronic bacterial infection that typically presents as a cutaneous lesion. Visceral involvement may occur, but mucosal disease is uncommon. We report two cases of sinonasal tract botryomycosis that clinically simulated a neoplasm. METHODS: Two cases of sinonasal tract botryomycosis were identified from the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology, Washington, DC. The clinical records, slides, and paraffin blocks were available for both cases. Histochemical stains, including Brown and Hopps, Gomori's methenamine-silver, acid-fast bacilli, mucicarmine, periodic acid-Schiff, and Warthin-Starry, were performed. RESULTS: The patients were an 81-year-old man and a 43-year-old woman. The man presented with acute ethmoiditis and a bulging eye. Radiographic studies showed a soft tissue mass in his left maxillary antrum with osseous erosion of adjacent anatomic sites. The woman presented with persistent headaches of more than 1 year's duration, with increasing severity in the months prior to presentation. An expansile soft tissue mass was identified in her right maxillary and ethmoid sinuses. Surgery was performed on both patients. The histology included amorphous, acellular material and separate, rounded eosinophilic granules associated with a neutrophilic infiltrate. A Splendore-Hoeppli phenomenon was seen. Filamentous gram-negative bacilli, identifiable only by histochemical staining, were morphologically compatible with Pseudomonas aeruginosa. Cultures of samples taken from both patients intraoperatively confirmed the organisms as P. aeruginosa. CONCLUSIONS: Sinonasal botryomycosis is a rare localized disease that may be mistaken clinically for an aggressive neoplasm. Complete surgical evacuation is curative.
Adult↗
Langerhans cell histiocytosis of the thyroid: a series of seven cases and a review of the literature.
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.
Adult↗
Olfactory neuroblastoma and other round cell lesions of the sinonasal region.
Sixty-nine round cell lesions of the sinonasal region (22 olfactory neuroblastomas [ONBs], 17 malignant lymphomas, nine Ewing's sarcomas [ES], nine rhabdomyosarcomas, three sinonasal undifferentiated carcinomas, five malignant melanomas, and four pituitary adenomas) were studied in an attempt to define the differential diagnostic capabilities of antibody to MIC2 and bcl-2 in paraffin-embedded tissue in the distinction of these lesions. In addition, antibody to p53 was applied in each case to define the incidence of p53 positivity among these various tumor types. Each of the ES cases was MIC2 positive; each of the other cases was MIC2 negative. Positivity for bcl-2 was confined to two cases, one of them a malignant lymphoma (85% of cells positive) and one an ONB (5% of cells positive). Small numbers of scattered p53-positive cells appeared in the majority of cases studied, without regard for the specific tumor type; only a single case, a malignant lymphoma, showed a majority (approximately 90%) of p53-positive cells. These results indicate that the MIC2 antibody is a useful method by which to distinguish ES from a variety of other round cell lesions that may be encountered in the sinonasal region. The practical applications of antibody to bcl-2 and p53 seem to be much more limited; by contrast, neither bcl-2 positive cells nor abundant p53 cells identified by immunohistochemical analysis seemed to be frequent findings in any of the tumor types studied. Although ONBs have been included with the peripheral primitive neuroectodermal tumors for classification purposes, these tumors diverge from the ES/primitive neuroectodermal tumor family in that they do not seem to share either the MIC2 positivity or the t(11;22) chromosomal translocation that typify the ES/primitive neuroectodermal tumor family of lesions. Although bcl-2 positivity has been associated with a light microscopic finding of an unfavorable histologic pattern in retroperitoneal neuroblastomas, it does not seem that bcl-2 positivity in ONB will select for a clinically distinctive subset of patients.
12E7 Antigen↗