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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rhabdoid tumor is a well-accepted clincopathologic entity among childhood renal neoplasms; similar tumors have been described in extrarenal locations. We present the clinicopathologic profile and the immunohistochemical features of a series of soft tissue rhabdoid tumors. Twenty-eight cases coded as extrarenal rhabdoid tumor (ERRT), RT, possible ERRT, and "large cell sarcoma" were retrieved from the Armed Forces Institute of Pathology soft tissue registry. The tumors were reclassified according to strict criteria by light microscopy, clinical information, immunohistochemistry, and, in some cases, electron microscopy. Soft tissue rhabdoid tumor (STRT) was defined as (1) a tumor composed of noncohesive single cells, clusters, or sheets of large tumor cells with abundant glassy eosinophilic cytoplasm, an eccentric vesicular nucleus, and an extremely large nucleolus; (2) positivity for vimentin and/or cytokeratin or other epithelial markers by immunostaining; and (3) exclusion of other tumor types with rhabdoid inclusions (melanoma, other sarcomas, carcinoma). Eighteen cases met our criteria for soft tissue rhabdoid tumors. The median patient age was 13 years (range, 6 months to 56 years). Ninety-four percent of STRT cases were positive for vimentin and 59% for pan-cytokeratin. Sixty-three percent and 60% were positive for CAM 5.2 and EMA, respectively. Seventy-nine percent stained for at least one epithelial marker; 76% stained for both vimentin and epithelial markers simultaneously. Forty-two percent stained for MSA, and 14% for CEA and SMA. CD99, synaptophysin, CD57 (Leu-7), NSE, and focal S100 protein were identified in 75%, 66%, 56%, 54%, and 31% of the STRT cases, respectively. All STRT cases examined were negative for HMB-45, chromogranin, BER-EP4, desmin, myoglobin, CD34, and GFAP. Follow-up examination in 61% of the STRT patients revealed that 64% of patients died of disease within a median follow-up interval of 19 months (range, 4 months to 5 years); 82% had metastases to lung, lymph nodes, or liver; 22% had local recurrences before metastasis; and 18% were alive without known disease status (median, 5.5 years). Soft tissue rhabdoid tumor is a highly aggressive sarcoma, predominantly of childhood. Besides having nearly consistent coexpression of vimentin and epithelial markers, STRTs show positivity for multiple neural/neuroectodermal markers that overlap with those of primitive neuroectodermal tumor.
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PMID:Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases. 993 May 72

Follicular dendritic cell sarcoma is a malignant tumor of the follicular dendritic cell which can arise in extranodal sites. We present here a case arising as a mass in the breast of a 41-year-old woman. The tumor was composed of mildly pleomorphic spindly cells with pale ovoid nuclei and cell processes intimately admixed with mature lymphocytes. In much of the lesion the cells were dispersed in cords in a myxoid stroma, and elsewhere there were solid sheets. The neoplastic cells were immunoreactive for CD21, CD35, EMA, and S100 protein, but not for other lymphoid markers or cytokeratin. Electron microscopy showed interdigitating cytoplasmic processes with junctions but no external lamina. The differential diagnosis includes carcinoma, lymphomas, and a variety of myxoid sarcomas. The tumor recurred within a few months and displayed increased nuclear pleomorphism and lymphatic invasion but the patient appears free of disease 3 years after the further excision. This case extends the spectrum of follicular dendritic cell sarcoma in soft tissue sites.
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PMID:Myxoid variant of follicular dendritic cell sarcoma arising in the breast. 1019 89

Heterotopic glial nodules occur most commonly in the head and neck area, and are theorized to arise following abnormalities in the development of the facial and skull bone plates. However, in spite of the fact that some of these lesions are associated with communication with the central nervous system (CNS), the lack of a meningeal component, argues against simple herniation and separation of brain tissue through a defect in the skull. We present an infant with a nodule directly over the spine present in the T-12 region with no skin abnormalities. Magnetic resonance imaging (MRI) and computerized axial tomography (CT) showed no spinal abnormalities with an overlying fibrotic soft tissue mass. The patient had no other associated clinical findings. Histologic findings showed a cellular component arising within the reticular dermis with a deep circumscribed margin. The nodule contained irregularly shaped cells containing abundant cytoplasm and indistinct cellular margins with bland nuclei. These cells were clustered around and between a fibro-mucinous stroma. Immunohistochemical stains showed positive staining for S-100 protein, vimentin, GFAP, NSE, and CD57, and negative staining for Ki-67, CD34, Neurofilament protein, cytokeratin, and EMA. The spindle cells showed positive staining for CD34 and vimentin. The clinical and histologic features and immunohistochemical profiles are used to separate this lesion from the closely related, ependymal rests, ependymomas, and primary cutaneous chorodomas.
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PMID:Glial heterotopia in the subcutaneous tissue overlying T-12. 1059 45

An exceptional case of malignant peripheral nerve sheath tumor with striated muscle differentiation and glandular component is reported, in a 52-year old man. This tumor measured 8 cm in diameter, and was localized in the chest wall, infiltrating the skeletal muscle. The mesenchymal portion of the tumor was composed mostly of spindle cells arranged in interlacing fascicles. Between these fascicles, there were large cells with abundant eosinophilic cytoplasm and clear elongated nucleus. Immunohistochemical study demonstrated cytokeratin, EMA and CEA expression in the glandular component and S100 protein expression in the major portion of the mesenchymal component. The large cells identified as rhabdomyoblasts, expressed desmin, myoglobin, alpha-SR actin and alpha-SM actin. The tumor recurred 5 years after its resection. Histological and immunohistochemical features were identical. We think that positivity of neoplastic striated muscle cells with alpha-SM actin reflects an early differentiation phase of these cells.
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PMID:[Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation and glandular component]. 1064 90

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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PMID:Sarcomatoid salivary duct carcinoma of the parotid gland. 1068 35

Malignant rhabdoid tumor (MRT), described for the first time in 1978 in the kidney, has rarely been reported in other organs including the brain and has involved adults in only 3 cases. We described a case of MRT in a 32-year-old woman who presented with severe headache, nausea and sudden onset of visual disturbance. MRI showed a well-enhanced mass at the suprasellar region. Subtotal removal of the tumor was performed. However, tumor regrowth occurred after the operation (doubling time, 8.36 days) and spinal dissemination was detected. Therefore, chemotherapy and radiotherapy were administered focusing on the suprasellar lesion and the spinal cord. Pathologically, light micrographs showed rhabdoid cells with large, round, single or double nuclei with one prominent nucleolus and eosinophilic cytoplasmic inclusions. Electron micrographs were made of typical rhabdoid cells displaying bundles of intermediate filaments within the perikaryon. In immunohistochemical studies, EMA, vimentin, cytokeratin and SMA were positive. Pathological findings were consistent with those of MRT. Optimal treatment for this tumor has not been established. Our case may be useful in defining treatment for MRT.
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PMID:[Suprasellar primary malignant rhabdoid tumor in an adult: a case report]. 1076 34

Among the histological variants of meningiomas the oncocytic subtype is rarely observed. Up-today, only six cases of oncocytic meningioma are described. This subtype of meningiomas shows an aggressive behavior and recurrences are more frequent. We describe a case of oncocytic meningioma in a 78-years-old woman. The patient had a history of breast cancer diagnosed 9 years before the brain biopsy; bilateral mastectomy and adjuvant chemotherapy was performed. She had a right frontal tumour measuring 3 cm in diameter. The patient is alive and well eleven months after surgery. The tumour was composed by large polygonal neoplastic cells with finely granular eosinophilic cytoplasm. Neoplastic cells were arranged in sheets and nests delimited by thin fibrous septa rich in vessels. Psammomatous bodies were also present. Mitoses were rare and necrosis was absent. Oncocytic differentiation was demonstrated by conventional histology and immunohistochemistry. Immunohistochemistry revealed a strong and diffuse positivity for antimitochondrial antiserum, vimentin and EMA; a focal reactivity for cytokeratin was observed. The rarity of oncocytic meningiomas is underlined with only six cases described in the world literature. The immunophenotypic profile and the differential diagnosis of the neoplasm is discussed and the concept of oncocytic meningioma as a distinct entity of tumour is emphasized.
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PMID:[Oncocytic meningioma. Case report]. 1083 73

A study to compare the immuno-histochemical profile of the human rete ovarii, and epoophoron, with the Fallopian tube and ovarian surface epithelium was performed with 31 antibodies and antisera. A reaction was present in the epithelial cytoplasm of the rete ovarii and epoophoron of mesonephric origin, for vimentin, GFAP, cytokeratin markers, (AE1/AE3, MNF116; Cam 5.2, 34 beta E12 and for the monospecific antibodies to cytokeratins 7 and 19), heat shock protein 27, in the cell membrane for HBME-1, EMA and in the subepithelial collagen for collagen IV. Reactions were present only in the epithelium in the rete ovarii for EGFR (one case) and CA-125 (four cases). A reaction was present in the epithelium of the epoophoron only for Ber-EP-4 and S100. There was no reaction with antibodies for desmin, neurofilament protein, cytokeratins 20 or 14, actin, calretinin, E-cadherin, C-erb-B2, or CEA (monoclonal and polyclonal reagents). The immuno-histochemical profile of the Fallopian tube was consistent with its para-mesonephric origin and that in the ovarian surface epithelium was consistent with a proposed modified mesothelial origin. This study provides an immunohistochemical profile of these structures with a large panel of commonly available antibodies and antisera, confirming and extending the findings described in previous studies.
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PMID:An immunohistochemical study of the rete ovarii and epoophoron. 1084 Aug 24

We report 15 primary renal neoplasms with morphologic, immunohistochemical, and molecular features identical to those of synovial sarcoma. These tumors form a distinct subset of the entity previously designated as embryonal sarcoma of the kidney. Most were diagnosed between the ages of 20 and 50 years. On gross examination, tumors are large, partially necrotic, and usually contain smooth-walled cysts. Microscopically, tumors are characterized by mitotically active, monomorphic plump spindle cells with indistinct cell borders growing in short, intersecting fascicles. Grossly identified cysts are lined by mitotically inactive polygonal eosinophilic cells with apically oriented nuclei ("hobnailed epithelium"). The spindle cells are immunoreactive for vimentin, often immunoreactive for EMA, but typically non-immunoreactive for desmin, actin, S100, or cytokeratins, whereas the cyst epithelium is cytokeratin-positive. These findings are consistent with monophasic, spindled synovial sarcoma encircling dilated native renal collecting ducts. The presence of an SYT-SSX gene fusion resulting from the t(X;18) characteristic of synovial sarcoma was demonstrated by reverse transcriptase polymerase chain reaction in three of three tumors in which adequate RNA could be obtained from paraffin blocks. An additional case demonstrated the characteristic t(X; 18) translocation on cytogenetic analysis, but adequate material to perform molecular studies was not available in this case or the remaining 11 cases. Primary renal synovial sarcoma is a distinctive clinicopathologic entity confirmed by molecular detection of SYT-SSX fusion transcripts.
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PMID:Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. 1093 49

Pericardial effusions are not uncommon in patients with an advanced malignancy Rarely malignancies may present initially with a pericardial effusion. Cytological examination of pericardial fluid may be valuable in differentiation of these cases. However, a metastatic tumour in serous effusion may not always show the functional differentiation of the primary tumour. In such a situation, although a wide range of special studies have been suggested for the diagnosis of malignancy we have found the use of a panel of a few common immunostains to be useful in confirming or suggesting the site of a primary tumour. The material for this study consisted of 76 pericardial fluids obtained between January 1991 and October 1998 from 46 males (mean age 59 years) and 30 females (mean age 52 years). Metastatic malignancy was diagnosed in 22 of the 76 patients and in 7/22 cases pericardial effusions were the initial presentation. The subsequent follow-up in the seven cases revealed adenocarcinoma of lung (n = 2), small cell anaplastic carcinoma of lung (n = 1), squamous cell carcinoma lung (n = 1), melanoma leg (n = 1), non-Hodgkin's lymphoma retroperitoneal lymph nodes (n = 1) and carcinoma of the breast (n = 1). Of the remaining 15 cases with a known history of malignancy, eight had cancers (three adeno; two small cell; one poorly differentiated, and two squamous cell types) of the lung; breast (n = 3); colon (n = 1); melanoma (n = 2) and non Hodgkin's lymphoma (n = 1). Immunostains which were useful in the diagnosis were EMA, CEA, cytokeratin, B72.3, HMB45, vimentin, S100, LCA, L26 and kappa and lambda light chains.
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PMID:The usefulness of a panel of immunostains in the diagnosis and differentiation of metastatic malignancies in pericardial effusions. 1101 58


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