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)

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

We report a case of brain surface clear cell ependymoma. A 13-year-old boy presented with complaints of right hypesthesia. Computed tomography and magnetic resonance image showed a left fronto-parietal cystic, calcified mass lesion. He underwent total resection of the tumor including cyst wall. The tumor located on the surface of the parietal lobe was sharply demarcated from the surrounding brain tissue and there was no continuity with the ventricular wall. Histological examination of the surgical specimens showed oligodendroglioma-like cells that had round unclei, clear cytoplasm which formed perivascular pseudorosettes, and immunoreactivity for glial fibrillary acidic protein (GFAP). Electromicroscopically, microvilli were seen. The findings were compatible with clear cell ependymoma. The cyst wall was lined with a layer of single cuboidal cells and, immunohistochemically, had no basal membrane. The inner surface of the cyst was positive for EMA, and the cuboidal cells were positive for GFAP. We discuss possible mechanisms for tumor growth in our case and the histogenesis of its cyst.
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PMID:[Brain surface clear cell ependymoma: case report]. 1047 46

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

A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. Immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.
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PMID:Meningioangiomatosis: a case report and review of the literature. 1061 86

A variety of cytomorphological features, architectural patterns and stromal changes may be observed in malignant melanomas. Hence, melanomas may mimic carcinomas, sarcomas, benign stromal tumours, lymphomas, plasmacytomas and germ cell tumours. Melanomas may be composed of large pleomorphic cells, small cells, spindle cells and may contain clear, signet-ring, pseudolipoblastic, rhabdoid, plasmacytoid or balloon cells. Various inclusions and phagocytosed material may be present in their cytoplasm. Nuclei may show bi- or multi-nucleation, lobation, inclusions, grooving and angulation. Architectural variations include fasciculation, whorling, nesting, trabeculation, pseudoglandular/pseudopapillary/pseudofollicular, pseudorosetting and angiocentric patterns. Myxoid or desmoplastic changes and very rarely pseudoangiosarcomatous change, granulomatous inflammation or osteoclastic giant cell response may be seen in the stroma. The stromal blood vessels may exhibit a haemangiopericytomatous pattern, proliferation of glomeruloid blood vessels and perivascular hyalinization. Occasionally, differentiation to nonmelanocytic structures (Schwannian, fibro-/myofibroblastic, osteocartilaginous, smooth muscle, rhabdomyoblastic, ganglionic and ganglioneuroblastic) may be observed. Typically melanomas are S100 protein, NKIC3, HMB-45, Melan-A and tyrosinase positive but some melanomas may exhibit an aberrant immunophenotype and may express cytokeratins, desmin, smooth muscle actin, KP1 (CD68), CEA, EMA and VS38. Very rarely, neurofilament protein and GFAP positivity may be seen.
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PMID:Morphological and immunophenotypic variations in malignant melanoma. 1079 80

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

Astroblastomas are uncommon brain tumors whose classification and histogenesis have been debated. Precise criteria for diagnosis have been described only recently, but have not found wide acceptance. We report the clinical, radiographic, and histopathologic features of 20 astroblastomas, and the chromosomal alterations in seven cases as detected by comparative genomic hybridization (CGH). The tumors occurred both in children and young adults (average age, 14 years), most often as well circumscribed, peripheral, cerebral hemispheric masses. Radiographically, the lesions were contrast-enhancing and solid, often with a cystic component. All were characterized histologically by astroblastic pseudorosettes, and most displayed prominent perivascular hyalinization, regional hyaline changes, and pushing borders in regard to adjacent brain. Tumor cells were strongly immunoreactive for S-100 protein, GFAP, and vimentin. Staining for EMA was focal. Ten of 20 astroblastomas were classified as "well differentiated" and 10 were classified as "malignant," largely on the basis of hypercellular zones with increased mitotic indices, vascular proliferation, and necrosis with pseudopalisading. All 10 well differentiated lesions and 8 of 10 malignant lesions were completely resected. None of the well differentiated astroblastomas recurred within the limited follow-up period. Three malignant astroblastomas recurred, including two incompletely resected tumors, and one that had been totally resected. One patient died of disease following recurrence. The most frequent chromosomal alterations detected by CGH were gains of chromosome arm 20q (4/7 tumors) and chromosome 19 (3/7). The combination of these gains occurred in three, including two well differentiated and one malignant astroblastoma. Other alterations noted in two tumors each were losses on 9q, 10, and X. These chromosomal alterations are not typical of ependymoma or infiltrating astrocytic neoplasms, and suggest that astroblastomas may have a characteristic cytogenetic profile in addition to their distinctive clinical, radiographic, and histopathologic features.
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PMID:Astroblastoma: clinicopathologic features and chromosomal abnormalities defined by comparative genomic hybridization. 1088 53

Surrounding the cells with a semipermeable polymeric membrane allows transplanting unmatched xenogeneic cells without a risk of their rejection. We prepared and tested several 2-hydroxyethyl methacrylate (HEMA) copolymers with alkyl methacrylates or acrylates to find out which was the most valuable for cell encapsulation. On the basis of optimum physical properties and good results of cytotoxicity tests, HEMA-EMA copolymer was chosen as a suitable candidate for encapsulation and immunoprotection of xenogeneic cells before their grafting into the central nervous system (CNS). To characterize the biocompatibility of p(HEMA-co-EMA) copolymer in the CNS, we implanted microcapsules made of this hydrogel into the brains of adult rats that were allowed to survive for 0.5, 1, 3, 6, and 9 months. Analysis of histological sections containing the implantation site was aimed at assessment of the cellular density at the implant-brain interface and identification of cell types participating in a tissue reaction. Our results indicated that the tissue reaction that was observed was caused largely by the implantation procedure because HLA-DR- and GSI-B4-positive macrophages/microglia infiltrated mainly the implantation channel. The number of these cells declined with time, which was true also for GFAP-positive reactive astrocytes, as well as for foreign body giant cells. The amount of connective tissue components surrounding the implanted microcapsules increased only slightly. These findings indicated that p(HEMA-co-EMA) hydrogel was well tolerated after implantation in the brain.
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PMID:Biocompatibility of HEMA copolymers designed for treatment of CNS diseases with polymer-encapsulated cells. 1102 87

Leiomyosarcoma is a relatively uncommon mesenchymal tumor that exhibits smooth-muscle differentiation. We report a new case of leiomyosarcoma involving the tongue of a 67-year-old male. Histologically, the tumor was composed of variably oriented fascicles of spindle-shaped cells with 'cigar-shaped' nuclei and eosinophilic cytoplasm, containing occasional PAS-positive granules. Atypical mitotic figures and necrotic foci were frequently detected. Consistent desmin, alpha-smooth muscle-specific and sarcomeric actin, and vimentin immunoreactivity was demonstrated in the tumor cells, whereas cytokeratins, CD 30, CD 31, CD 34, CD 45, CD 68, EMA, GFAP, HMB 45 and S-100 protein were negative. The patient underwent wide surgical excision of the tumor and is alive and disease-free at a 5-year follow-up. This report emphasizes the difficulties in the differential diagnosis of these uncommon tumors in an intra-oral location.
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PMID:Primary intraoral leiomyosarcoma of the tongue: an immunohistochemical study and review of the literature. 1103 45

Epithelioid schwannomas occur predominantly in relation to peripheral nerves and are associated with histological and clinical malignancy. However, a variant of the epithelioid schwannoma involving cranial nerves is extremely rare. In this study we report three cases of epithelioid schwannomas originating from the acoustic nerves and located in the cerebello-pontine angles. In the first case, the tumor was histopathologically entirely solid and demonstrated biphasic pattern with both spindle-shaped cells and a population of round or polygonal epithelioid cells. The second one consisted of the smaller part exhibiting typical Antoni B and A tissue and large areas containing clusters and bundles of epithelioid cells. Purely epithelioid schwannoma composed predominantly of cords or nests of round and polygonal epithelioid cells were observed in the third case. All schwannomas revealed marked polymorphism and nuclear hyperchromasia. Immunohistochemical studies showed a diffuse, strong positivity for S-100 protein in the cytoplasm of the spindle and epithelioid tumor cells. These two populations of cells were positively stained for vimentin, but were negative for EMA, cytokeratin and HMB45. Patchy GFAP-immunoreactivity was also noticed at the peripheral parts of the tumors. The authors discuss differential diagnosis of this unusual variant of schwannoma in relation to malignant transformation of the epithelioid component.
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PMID:Epithelioid schwannomas of the acoustic nerve. 1105 30


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