Cases reported "Sarcoma"

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1/54. Sarcomatoid salivary duct carcinoma of the parotid gland.

    Salivary duct carcinoma (SDC) is a high-grade neoplasm known to histologically resemble high-grade ductal carcinoma in situ of the breast. We describe 3 cases of sarcomatoid salivary duct carcinoma, a heretofore unreported variant of SDC. Each case was a composite of SDC and sarcomatoid carcinoma and histologically similar to reported cases arising in the breast. The clinicopathologic features, including immunohistochemistry, of 3 cases were investigated. In the 3 men, ages 56, 68, and 70 years, the resected parotid tumors measured 1.5, 3.5, and 1.5 cm, respectively. Only the 3.5-cm tumor extended beyond the parotid gland into soft tissue. This patient died at 3 years with pulmonary metastases. The other patients were free of disease at 6 and 12 months. Histologically, each case was a composite of usual-type SDC and sarcomatoid carcinoma. SDC showed typical cribriform architecture, whereas anaplastic, spindled cells constituted the sarcomatoid areas. Immunohistochemically, epithelial elements stained as follows: cytokeratin (AE1/AE3 & CAM 5.2) positive in 3 of 3 cases, EMA positive in 3 of 3 cases, vimentin negative in 3 of 3 cases, desmin negative in 3 of 3 cases, c-erbB-2 positive in 1 of 2 cases. Sarcomatoid elements stained as follows: AE1/AE3 negative in 3 of 3 cases, CAM 5.2 rare positive cell in 1 of 3 cases, EMA focally positive in 3 of 3 cases, vimentin positive in 3 of 3 cases, desmin negative in 3 of 3 cases, c-erbB-2 negative in 2 of 2 cases. Electron microscopy, performed in one case, showed scattered junctional complexes congruent with epithelial differentiation. Immunohistochemical results, EMA and CAM 5.2 positivity, and ultrastructural findings supported our belief that these unique biphasic tumors represented SDC with sarcomatoid carcinoma. We conclude an element of sarcomatoid carcinoma rarely may arise in association with SDC, and it is erroneous to diagnose such tumors as "carcinosarcoma."
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2/54. Immunoblastic sarcoma with features of sjogren's syndrome and systemic lupus erythematosus in a patient with immunoblastic lymphadenopathy.

    A patient with immunoblastic lymphadenopathy and features of sjogren's syndrome and systemic lupus erythematosus is presented. Clinical features included generalized lymphadenopathy, rash, alopecia, and synovitis, with associated laboratory abnormalities of a positive antinuclear factor and double-stranded dna antibodies, positive lupus band test, and hemolytic anemia. Symptoms of sjogren's syndrome included dry eyes and mouth and swollen parotid gland; biopsy results of the accessory salivary glands were positive. At autopsy immunoblastic sarcoma was found that involved the myocardium, which stained for both kappa and lambda light chains by immunoperoxidase techniques.
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3/54. Myxoid liposarcoma of the oral cavity with involvement of the periodontal tissues.

    BACKGROUND, AIMS: liposarcoma is the 2nd most frequent soft tissue sarcoma in adults, but it is extremely rare in the head and neck and, particularly, in the oral cavity. We report on a 25-year-old female who presented with a periodontal mass, extended from the right upper 3rd molar to the right upper 2nd premolar, covered by intact oral mucosa. The clinical differential diagnosis included peripheral giant cell granuloma, salivary gland neoplasms, squamous cell carcinoma of the gingiva, sarcoma and malignant lymphoma. methods: To accurately plan subsequent treatment, an excisional biopsy was performed and a myxoid liposarcoma was diagnosed. Consequently, the patient underwent wide excision of the neoplasm with maxillary en-block resection. RESULTS: The post-operative course was uneventful and the patient is alive and well 8 years after the original diagnosis. The authors stress the importance of considering soft tissue sarcomas in the diagnostic approach to patients with unusual periodontal neoplasms and to plan adequate surgical sampling of the lesion (i.e. excisional biopsy). CONCLUSIONS: This appears of pivotal importance as more limited specimens may result in inaccurate pre-operative diagnosis.
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4/54. Malignancy of the parotid gland with primary symptoms of a temporomandibular disorder.

    The purpose of this case report is to highlight the possibility that malignant neoplasms of the deep lobe of the parotid gland may present as a temporomandibular disorder (TMD). Two patients who were eventually diagnosed with deep-lobe parotid tumors were retrospectively analyzed clinically, radiographically, and pathologically. For the first patient, there was a prolonged delay in establishing the correct diagnosis; for the second patient the delay was shorter. This was done primarily by computed tomographic examination. Despite aggressive surgical, radiotherapeutic, and chemotherapeutic treatment, both patients died from their malignant disease. All clinicians who treat TMD must be aware of the rare possibility of deep-lobe parotid tumors presenting as a TMD.
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5/54. Malignant eccrine spiradenoma: a case report and review of the literature.

    BACKGROUND: Eccrine spiradenoma is a well-differentiated benign tumor of the sweat glands. Malignant change arising within eccrine spiradenoma is rare. OBJECTIVE: We describe a patient with malignant eccrine spiradenoma exhibiting both carcinomatous and sarcomatous differentiation. methods: Case report and literature review. RESULTS: A 37-year-old woman noted enlargement of a left axillary tumor that had been present for 20 years. The tumor was resected and the specimen, measuring 3.0 cm x 1.5 cm, revealed an encapsulated benign eccrine spiradenoma as well as an undifferentiated carcinoma possessing both carcinomatous and sarcomatous components. A transition zone was evident between the benign eccrine spiradenoma and the undifferentiated carcinoma, suggesting that the latter had arisen from the benign tumor. The malignant areas consisted principally of undifferentiated carcinoma (70%), although squamous cell carcinoma (10%), adenocarcinoma (10%), and chondrosarcomatous (10%) components were also present. Numerous mitotic figures were noted within the areas of malignant change, suggesting that the tumor was aggressive in nature. The patient died of systemic metastases 7 months after diagnosis. CONCLUSION: Although eccrine spiradenomas are usually benign, they can, on rare occasions, undergo malignant transformation. This case report describes one such occurrence of malignant transformation of a benign eccrine spiradenoma that unfortunately resulted in the patient's death from systemic metastases 7 months after diagnosis.
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6/54. Undifferentiated sarcoma of the parotid gland with osseous metaplasia.

    Malignant spindle cell tumors of the parotid gland are a diagnostic challenge. We present an unusual case of such a tumor that occurred in the right parotid gland of a 53-year-old man. The clinical and histologic assessments were consistent with a primary sarcoma of the parotid gland. The tumor was composed of sheets of pleomorphic, spindle-shaped cells with an area of bone formation. By immunohistochemistry, the tumor cells were positive for vimentin and negative for epithelial markers. Electron microscopy revealed mesenchymal cells containing moderate amounts of rough endoplasmic reticulum. The major differential diagnostic considerations were spindle cell carcinoma, carcinosarcoma, and primary undifferentiated sarcoma with osseous metaplasia. The lack of epithelial features and the benign appearance of the bone formation led to a diagnosis of undifferentiated sarcoma of the parotid gland.
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7/54. Sarcoma of the thyroid gland: a case report.

    Sarcoma of the thyroid gland is a very uncommon malignant tumor (<1% of thyroid cancers) characterized by severe local course and rapid metastatic dissemination and very poor prognosis. We report the case of an 84-year-old woman hospitalized for expiratory dyspnea, severe malnutrition and swallowing disorders leading to bronchial infection. She had a voluminous (20 cm) extra-thoracic right-neck goiter with a considerable superficial venous pattern that had developed on an old goiter before growing recently. CEA and calcitonin levels were normal. The cervical-thoracic CT demonstrated a partially necrosed heterogeneously hypodense tumor compressing the esophagus and the trachea with displacement of adjacent structures which were not directly invaded. Large areas of necrosis and a probable metastatic image measuring 3 cm in the right median pulmonary lobe were observed. Macrobiopsy disclosed grade 3 sarcoma. Complete resection delivered a 3.170 kg tumor. histology confirmed the diagnosis of sarcoma without neoplastic extension. Apart from right recurrent palsy, the initial post-operative period was satisfactory and the patient was discharged. Four and a half months later she was rehospitalized with local recurrence with a large metastatis in the right lung. She died two weeks later.
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8/54. Occult metastatic follicular thyroid carcinoma masquerading as a soft tissue sarcoma of the gluteal region.

    This is a case of an occult follicular thyroid carcinoma in a 61 year old civil servant presenting with bony metastasis to the left iliac bone twenty years after an initial subtotal thyroidectomy. There was a soft tissue mass with associated cup shaped Iytic detruction of the iliac bone and on ultrasound scan the mass was found to be of mixed echogenicity and areas of sonolucencies which were due to necroses. At autopsy, the thyroid gland appeared macroscopically within normal limits but histology confirmed Follicular Thyroid Carcinoma.
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9/54. Soft tissue myoepithelioma: a case report.

    Myoepitheliomas are tumours composed predominantly or exclusively of myoepithelial cells. They are well described, especially within the salivary gland, but their occurrence in soft tissues is less well known and this often results in diagnostic problems. We report a case involving the deep soft tissues of the lower neck behind the clavicle. Grossly, the tumour was well circumscribed with solid and cystic areas. histology showed a richly vascularised tumour composed of bland round, ovoid or spindle-shaped cells. Various growth patterns were present including solid, nested, microcystic and trabecular arrangements. In some areas there was an alveolar pattern with tumour cells lining fibrous septae. immunohistochemistry showed diffuse strong positivity for S100 protein, calponin, vimentin and glial fibrillary acidic protein and focal positivity for epithelial membrane antigen, 34betaE12 and AE1/AE3, in keeping with myoepithelial differentiation. Electron microscopy revealed tumour cells surrounded by basal lamina with subplasmalemmal densities and containing cytoplasmic myofilaments. This case report highlights the rare occurrence of myoepitheliomas in deep soft tissues. Pathologists should be aware of this and should consider a myoepithelioma in the differential diagnosis of a soft tissue spindle cell neoplasm.
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10/54. Fine-needle aspiration cytology of extranodal natural killer/T-cell lymphoma.

    Extranodal NK/T-cell lymphoma, nasal type, is a predominantly extranodal lymphoma characterized histologically by prominent necrosis, angiocentric growth, and vascular destruction. Only one report describing its fine-needle aspiration (FNA) cytologic features is available and shows highly unusual findings for a lymphoma. The present case concerns a 58-yr-old patient that presented with a soft tissue mass of the thigh in addition to an ulcerative lesion of the palate and nodular hepatic and splenic lesions. FNA cytology of the thigh tumor was interpreted as a malignant mesenchymal lesion (sarcoma). The subsequent pathologic study revealed an NK/T-cell lymphoma. Our findings are very similar to those previously reported. They were highly unusual for a lymphoma and consisted of polymorphic, round to spindle neoplastic cells distributed in irregular aggregates, and single cells. No significant number of lymphoglandular bodies were present.
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