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1/27. Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid with prominent mitotic activity and focal necrosis.

    Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare, apparently low-grade spindle cell tumor of the thyroid gland occurring in young individuals and thought to be derived from thymic or branchial pouch remnants. Spindle epithelial tumor with thymus-like differentiation has little to no mitotic activity, and focal necrosis has been reported in one case. We present a case of SETTLE in a 29-year-old man that was initially evaluated by fine-needle aspirate biopsy and ultimately found to be consistent histologically with SETTLE. In this case, there were numerous mitotic figures among the spindle cells and focal necrosis. Spindle epithelial tumor with thymus-like differentiation has been considered to be a tumor of low malignant potential with metastases developing some years after diagnosis. This is the first case in which prominent mitotic activity and necrosis is reported perhaps representing an aggressive variant.
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2/27. Sialoblastoma and epithelial tumors in children: their morphologic spectrum and distribution by age.

    This commentary addresses the histologic spectrum of salivary gland neoplasms in children with emphasis on perinatal tumors. Histopathologically, perinatal tumors fall into four categories: 1) histologically benign with adult counterpart, 2) hamartomas 3) embryomas-sialoblastomas, and 4) histologically and biologically malignant adult analogue tumors. Although the criteria to serrate benign from malignant sialoblastomas are not well-established, the following histologic features would favor an aggressive clinical course: perineural and/or vascular spaces invasion, necrosis, and a cytologic atypia beyond the expected for embryonic epithelium.
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3/27. Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid.

    BACKGROUND: Tumors of the so-called intrathyroidal epithelial thymoma type are a rare group of thyroid neoplasm tumors. Of this type of tumor, spindle epithelial tumor with thymus like differentiation (SETTLE) has been reported only 17 times in English literature. methods: An 18-year-old woman was initially seen with a 6-cm left thyroid mass that was resected with a left thyroidectomy. Histopathologic features of the excised left thyroid tumor together with an immunohistochemistry and electron microscopy led us to a diagnosis of SETTLE. RESULTS: Most of the specimen was composed of highly cellular spindle cells that formed intersecting and streaming fascicles. The spindle cells showed both vimentin immunoreactivity and cytokeratin immunoreactivity but no immunoreactivity for thyroglobulin or calcitonin. Electron microscopic examination of the spindle cells demonstrated prominent cytoplasmic tonofilaments, desmosomes, and basal lamina consistent with epithelial cell origin. dna content analysis by flow cytometry revealed dna diploidy. CONCLUSIONS: SETTLE of thyroid gland is an extremely rare entity. A review of the literature reveals that SETTLE has distinctive morphologic features and an immunohistochemical profile.
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4/27. Glandular patterns in a thyroid carcinoma with insular and anaplastic features: a case with possible implications for the classification of thyroid carcinomas.

    We describe the case of a 33-year-old woman with a thyroid carcinoma showing poorly differentiated (insular), anaplastic, and glandular features, the latter with extensive clear cell changes. Grossly, the well-circumscribed tumor nodule measured 3.6 cm in maximum dimension and was confined to the thyroid. Microscopically, the majority of the tumor was composed of well-defined "insular" nests showing microfollicular formation, high mitotic activity, and areas of necrosis. Other regions, as well as the intervening stroma of the insular nests, were characterized by highly atypical and pleomorphic stromal cells, extensive necrosis, and malignant cartilaginous nodules. Approximately 30% of the tumor was composed of diffuse glandular formations, each of which were lined by elongated, simple columnar cells with basally situated, mildly pleomorphic nuclei, clear supranuclear, periodic acid-Schiff (and diastase sensitive) cytoplasm, empty lumens, and no myoepithelia or basement membranes. Immunohistochemically, the glandular elements displayed diffuse and strong positivity for thyroid transcription factor-1, bcl-2, and CAM 5.2, sparse positivity for thyroglobulin and Ki67, and diffuse but weak positivity for p53. calcitonin was negative throughout the tumor. Karyotypic analysis of a primary culture showed a complex hypertriploid karyotype including structural abnormalities of chromosomes X, 1, 4, 6, 9, 13, and 14 in the majority of cells examined. This composite of histologic findings, especially the glandular patterns, is unusual and their prognostic significance is unclear. The patient is alive with no evidence of tumor recurrence or metastasis at 5 months follow-up. overall, the morphologic and immunohistochemical properties of the glandular component suggests that they are less differentiated than well-differentiated carcinomas and are probably more differentiated than the insular component. This case supports the theory that the various primary carcinomas of the thyroid may represent points along a spectrum rather than distinct entities.
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5/27. Malignant peripheral nerve sheath tumor with divergent differentiation.

    Right posterior thigh malignant peripheral nerve sheath tumor with malignant glandular and rhabdomyoblastic components was diagnosed in a 24-year-old African American man. Malignant glands and rhabdomyoblastic cells were admixed with the spindle cells. immunohistochemistry demonstrated spindle cells positive for S100 and vimentin; the glandular component was positive for chromogranin, and rare cells were positive for CK20 but negative for CK7. Rhabdomyoblastic cells were positive for muscle-specific actin and desmin. A single pulmonary metastasis occurred 5 months after surgical resection of the tumor. death occurred 1 month later and was probably due to a pulmonary embolus.
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6/27. Epithelial-myoepithelial carcinoma of the lung. A case report and review of the literature.

    Primary lung tumors mimicking the salivary gland-type neoplasms are extremely rare. These types of tumors originate from submucosal bronchial glands. Epithelial-myoepithelial carcinoma is an uncommon tumor in this group, and only 7 cases have been reported. It is considered to be a low-grade malignant neoplasm. We report a case of epithelial-myoepithelial carcinoma of bronchial gland origin in a 73-year-old man who presented with coughing and dyspnea. The bronchus of the left lower lobe contained a tumor mass that had a polypoid growth pattern. The tumor also extended into the pulmonary parenchyma, forming a well-circumscribed mass with a pushing margin. The tumor consisted of epithelial and myoepithelial cells. The epithelial cells were positive for cytokeratins and epithelial membrane antigen, while the myoepithelial cells were positive for S100 protein and muscle-specific actin. According to these findings, we diagnosed epithelial-myoepithelial carcinoma. After undergoing pneumonectomy, the patient has been disease free for 34 months.
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7/27. Cardiac myxoma with glandular elements: a histologic, histochemical, and immunohistochemical evaluation.

    Epithelial differentiation in cardiac myxoma is a rare phenomenon. Out of 104 surgically excised specimens, we studied 3 cases of cardiac myxoma with glandular differentiation. All the cases had well formed glands in addition to the myxoma cells lying in a myxoid background. Detailed histochemical and immunohistochemical studies suggest that the epithelial islands in cardiac myxoma show an enteric phenotype.
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8/27. Sialoblastoma: a case report and review of the literature on congenital epithelial tumors of salivary gland origin.

    The histologic, immunohistochemical, and ultrastructural features of a congenital epithelial tumor of the parotid were studied. The tumor was characterized by solid nests of epithelial cells intermingled with proliferating ductal structures lined by a double layer of cells. Immunoperoxidase staining for cytokeratin, vimentin, actin, and S-100 protein showed the presence of cytokeratin in the ductal cells as well as the presence of vimentin, actin, and S-100 protein in the outermost layer of the ducts. The solid nests were focally reactive to S-100 and vimentin. Ultrastructural examination revealed myoepithelial cells with replication of basement membrane material. The tumor recurred 17 months after excision without lymph node involvement or metastasis. The term "sialoblastoma" is favored. review of the literature on congenital, epithelial salivary gland tumors showed that a few cases recurred locally and only one case had regional lymph node involvement. No distant metastasis has been reported.
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9/27. Imprint cytology of metastatic sialoblastoma. A case report.

    BACKGROUND: Sialoblastoma is a rare, aggressive and potentially malignant perinatal/congenital tumor that recapitulates the developing salivary gland. There is only 1 brief description of the cytologic findings of metastatic sialoblastoma and 1 poorly documented case of lung metastasis in the literature. CASE: A 75-month-old girl with a history of recurrent sialoblastoma initially diagnosed at 21 months and treated with multiple incomplete surgical excisions, chemotherapy and radiation presented with a solitary lung nodule. Imprint smears and frozen section of the mass were diagnostic of metastatic sialoblastoma. CONCLUSION: Cytologic features of sialoblastoma showed complete concordance with histology and included the presence of variably arranged, tight, solid clusters of atypical-appearing, basaloidlike cells in a background of dispersed epithelial and myoepithelial cells. The clusters contained admixed benign ductal cells and dense, metachromatic, magenta hyaline globular material with smooth, rounded outlines. The differential diagnoses include neoplasms composed of either basaloid cells and/or admixed hyaline matrix material and included pleomorphic adenoma, basal cell adenoma and adenoid cystic carcinoma. All these neoplasms affect patients in the first 2 years of life, whereas sialoblastoma usually occurs in the first 2 decades of life.
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10/27. Low-grade renal epithelial tumor originating from the distal nephron.

    There are few published reports of low-grade renal epithelial tumor originating from the distal nephron. However, it should not be disregarded clinically, because the actual number of patients with such tumors may be higher than expected. We investigated the immunohistochemical profile of a histologically distinct subtype of such a tumor in detail, in addition to the clinical course and imaging studies. The present study demonstrated that both glandular and spindle cell components of this tumor have a persistent characteristic of an epithelial tumor arising from the distal tubule or collecting duct. This tumor is a benign complex neoplasm that can be treated successfully with radical surgery. Beta-catenin and E-cadherin are suggested to play a crucial role in tumorigenesis and the biphasic arrangement of this neoplasm, concerning the expression of epithelial membrane antigen and carbohydrate antigen 19-9. We suggest that the term 'distal nephron epithelioma' is appropriate for classifying such rare but clinicopathologically distinct tumors.
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