Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a case of a sarcoma arising in the hyaline-vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38-year-old man who clinically presented with a subcutaneous non-fixed cyst-like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle-cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HVCD, whereas the spindle-cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous component was diffusely positive for fascin, nerve growth factor receptor, and CD34 with focal weak reactivity for CD21 and CNA.42. Stains for CD23, CD31, CD35, CD99, ALK-1, SMA, ASMA, desmin, factor XIIIa, AE1-AE3, EMA, bcl-2, S-100, Melan-A, HMB-45, Cam 5.2, and factor VIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocytes was detected and we could not identify EBV or HHV-8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth-like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.
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PMID:Sarcoma arising in hyaline-vascular castleman disease of skin and subcutis. 1612 Oct 55

Mucinous tubular and spindle cell carcinomas are low-grade renal epithelial neoplasms, which were first recognized as a specific entity in the World Health Organization 2004 classification. Forty-five documented cases have been reported. We present 15 additional cases that were incidentally discovered in ten women and five men, with a mean age of 53 years. The tumor is characteristically made up of large eosinophilic regular spindle cells separated by a myxoid stroma with intercellular alcian-blue-positive clear droplets. In peripheral areas, elongated tubules and papillae covered by cubic cells are found. Until this entity had been defined, pathologists used to classify these tumors as variants of solid papillary carcinomas with compressed and elongated papillae, metanephric adenomas, and sarcomatoid carcinomas. In the literature, cytogenetic data indicate various chromosomal losses and gains, but no loss of 3p or trisomy 7 and/or trisomy 17. In two cases, we demonstrate chromosomal loss involving chromosomes 1, 4, 6, 11, 8, 13, 14, 15, 18, and 22. In our 15 cases, immunohistochemistry favored a distal tubule origin (EMA(+), AE1/AE3(+), CK7(+), CK19(+), E-cadherin(+), AMACR(+), and CD10(-)). Prognosis was favorable in our cases, while in the literature, two metastatic cases were reported. Further investigations are required to determine the frequency and true prognosis of these tumors, which are easily identifiable morphologically.
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PMID:Mucinous tubular and spindle cell carcinoma: a report of 15 cases and a review of the literature. 1623 Nov 79

A case of a mature adult teratoma of the right testicle in 34-year-old man is reported. Microscopically, well-differentiated gut and respiratory epithelium prevailed in the lesion. There were also epidermis and well-developed skin adnexa. In addition, a focus of a meningothelial cell proliferation containing psammoma bodies was detected. There were also a peripheral nerve and glial tissue adjacent to the meningothelial elements. The meningothelial cells were immunohistochemically positive for EMA and claudin and negative for S-100-protein, cytokeratins (AE1-AE3, CAM5.2), desmin, and chromogranin A. The glial tissue was positive for
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PMID:[Testicular teratoma with meningothelial elements]. 1632 80

The aims of our paper were to establish the main histopathological, histochemical and immunohistochemical aspects of tumoral stroma from salivary pleomorphic adenomas. For this purpose we investigated 103 cases by the classical histopathological technique with paraffin embedding and staining with Hematoxylin-Eosin (HE), Hematoxylin-Eosin-Safranin (HES), trichromic Masson, trichromic Goldner Szeckelly, orcein and Periodic Acid Schiff-Blue Alcian (PAS-AA). Immunohistochemically, they were investigated for AE1-AE3, MNF116, CK8, EMA, vimentin, alpha-actin calponin, S-100, GFAP, collagen IV, and PCNA. The results of our study suggest the key role of neoplastic myoepithelial cell in the achievement of diverse morphological aspects of stroma in such neoplasms.
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PMID:Tumoral stroma of salivary pleomorphic adenoma -- histopathological, histochemical and immunohistochemical study. 1644 8

We report a case of an aldosterone producing adrenocortical adenoma with rhabdoid features in a 16-year-old girl. Grossly, the tumor measured 30 mm in diameter and weighed 24 g. Histologically, the tumor was composed of approximately equal parts of tumor cells with rhabdoid features arranged in a solid and trabecular pattern and cells characterized by compact eosinophilic cytoplasm, solid growth with focal necroses, and increased mitotic activity. The lipid-rich tumor cells with ample clear vacuolized cytoplasm represent a minor component. Immunohistochemically, all the tumor cells showed the same results and were positive for vimentin, synaptophysin, Melan A, and alpha-inhibin. Cytokeratin CAM 5.2 was positive only focally. Chromogranin A, actin, alpha-actin, S100 protein, EMA, and cytokeratin AE1/AE3 were negative. Rhabdoid features have been described in many tumors of variable histogenesis; however, to the best of our knowledge, the presence of rhabdoid phenotype has never been described in either adrenocortical adenoma or carcinoma.
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PMID:Adrenocortical adenoma with rhabdoid features. 1644 85

We report four previously undescribed primary giant cell tumors of the extrahepatic biliary tree and morphologically compare them with 10 anaplastic spindle and giant cell carcinomas with osteoclast-like giant cells of the gallbladder. Two giant cell tumors were located in the distal common bile duct; one in the cystic duct and one in the gallbladder. The 3 patients with bile duct tumors were male, and the only patient with a gallbladder tumor was a female. The age of the patients ranged from 45 to 60 years with an average of 55 years. The patients with bile duct tumors presented with biliary obstruction, and the patient with a gallbladder tumor presented with symptoms of cholelithiasis and a gallbladder mass. Histologically, the tumors were similar to giant cell tumors of bone. They consisted of a mixture of mononuclear and multinucleated osteoclast-like giant cells. The mononuclear cells showed no atypical features, and their nuclei were similar to those of the multinucleated giant cells. CD163 immunoreactivity was restricted to the mononuclear cells, whereas CD68 and HAM 56 labeled only the multinucleated osteoclast-like giant cells. The mononuclear cells were EMA-positive but did not express cytokeratins. Follow-up showed that 3 patients were alive and disease-free 3.7 to 7 years after surgery. The anaplastic spindle and giant cell carcinomas contained a fewer number of osteoclast-like giant cells, and their mononuclear cells showed considerable variation in size and shape, marked cytologic atypia, and numerous mitotic figures. They were focally cytokeratin positive (AE1/AE3; CAM 5.2) and did not label with CD163, CD68, and HAM 56. The benign osteoclast-like giant cells showed immunoreactivity for CD68 and HAM 56 but were negative for CD163 and cytokeratins. Giant cell tumors of the extrahepatic biliary tree are benign true histiocytic neoplasms that should be distinguished from the highly lethal anaplastic spindle and giant cell carcinomas with osteoclast-like giant cells by detailed cytologic analysis and immunohistochemical stains for CD163, CD68, HAM 56, and cytokeratins.
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PMID:Giant cell tumor of the extrahepatic biliary tree: a clinicopathologic study of 4 cases and comparison with anaplastic spindle and giant cell carcinoma with osteoclast-like giant cells. 1822 38

Papillary renal cell carcinoma (RCC) is subclassified in type 1 displaying cells with scanty pale cytoplasm arranged in a single layer and in type 2 showing pseudostratified cells with eosinophilic cytoplasm. However, the existence of more variants of papillary RCC may be inferred by the recognition of few cases with different morphological features. We report the clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic features of 12 papillary RCC composed by oncocytes. Ten patients were males and their median age was 67 years. The tumors were well demarcated and their median diameter was 7.1 cm. Solid oncocytoma-like areas occurred in 11 cases. The cytoplasm of the neoplastic cells was filled by mitochondria with lamellar cristae. All cases were positive for the antimitochondrial antigen and racemase and showed variable immunoreactivity for cytokeratins (AE1/AE3, CK8-18, CK7, CK19), EMA, CD10, vimentin, and parvalbumin. MIB1 was detected in 0 to 6 cells per 1 high-power field. Fluorescent in situ hybridization analysis on formalin-fixed paraffin-embedded tissue showed three or more signals for chromosome 7 and 17 (for both > or =30% of nuclei in 7 of 12 neoplasms). In males, signals of chromosome Y were absent in more than 80% of the neoplastic nuclei. One patient died of metastases. Interphase cytogenetic analysis by fluorescent in situ hybridization can be a diagnostic tool in cases mimicking an oncocytoma.
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PMID:Oncocytic papillary renal cell carcinoma: a clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic study of 12 cases. 1673 Mar 6

Synovial sarcoma (SS) is an uncommon soft tissue tumor occurring mainly in the periarticular region of the extremities of young adults. In this report, we describe a very rare occurrence of primary SS of the esophagus in a 72-year-old woman. Histologically, the tumor demonstrated biphasic morphologic findings associated with poorly differentiated areas. Tumor cells expressed vimentin, epithelial (EMA, CK7, AE1/3), bcl-2 and neuroectodermal (CD56, CD57, CD99) antigens. Differential diagnose included esophageal sarcomatoid carcinoma. Cytogenetic analysis confirmed the diagnosis of SS by identifying t(X;18) translocation. The literature of this very uncommon entity of the esophagus is reviewed.
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PMID:Diagnosis of primary esophageal synovial sarcoma by demonstration of t(X;18) translocation: a case report. 1678 28

A 7-year-old boy presented with a 1-year history of back pain radiating to his left scapula and arm, with tenderness to palpation over the area. Laboratory studies were unremarkable. Computed tomographic scan showed a mixed lytic and sclerotic process at the T5-T6 level of the vertebral column in continuity with an adjacent paravertebral soft tissue mass. Nuclear medicine scan demonstrated increased uptake in the T5 vertebral body. Histology revealed invasive squamous cell carcinoma infiltrating mature, gliotic neural tissue, with areas of necrosis and prominent perivascular space involvement. Associated vertebral fragments showed bone destruction, reactive bone formation, and fibrosis. By immunohistochemistry, the carcinoma cells were positive for cytokeratin AE1/AE3, cytokeratin 5/6, EMA, and MIC-2 (membranous staining). The mature neural tissue was positive for GFAP; immature neural elements were not identified. Based on morphologic, immunohistochemical, and clinical features, this lesion was diagnosed as invasive squamous cell carcinoma arising within an intravertebral and paravertebral teratoma.
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PMID:Squamous cell carcinoma arising in a pediatric intra- and paravertebral teratoma. 1694 83

4 cases (2 males and 2 females) of mucinous tubular and spindle-cell carcinoma of the kidney were studied. The patients' age was 43 to 87 years. Microscopically, this tumor comprised two major cell populations. One of them involved spindle cells with a sparse cytoplasm, which were arranged in the fascicular pattern, often resembling low-grade smooth muscle tumors. The other population was small cuboidal cells with round nuclei and an eosinophilic cytoplasm. The mitotic activity was low. Only one tumor had both cell components. The other three tumors were represented by the second type cells. Immunohistochemical staining with avidin-biotin was performed by using cytokeratin, vimentin, SMA, p53, Bcl 2, EMA, and CD10. All cases were stained positively with antibodies to the cytokeratins AE1-AE2 and vimentin. The expression of EMA, Bcl 2, and CK7 was varying in different tumors. Responses to smooth muscle actin, p53, CK20, and CD10 were negative in all cases. Mucinous tubular and spindle-cell carcinoma of the kidney should be differentiated from angiomyolipoma with the preponderance of a leiomyomatous component, renal smooth muscle neoplasms, metanephrotic adenoma, and renal cell carcinoma with sarcomatoid differentiation.
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PMID:[Mucinous tubular and spindle-cell carcinoma of the kidney]. 1698 88


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