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)

Low-grade fibromyxoid sarcomas (LGFMS) bear either the t(7,16) (q32-34;p11) or t(11,16) (p11;p11) translocations, resulting in FUS-CREB3L2 or FUS-CREB3L1 fusions, respectively. Heretofore, fusion transcripts were mainly detected in frozen tissues, using reverse transcription-polymerase chain reaction. In this study, we aimed to develop a reliable method to detect these in paraffin-embedded tissues, and to examine the clinicopathologic characteristics of a series of translocation-positive LGFMS. Sixty-three neoplasms with typical morphologic features of LGFMS and 66 non-LGFMS tumors selected for their resemblance to LGFMS (LGFMS-like tumors) were examined. RNA of sufficient quality could be extracted from 111/129 (86%) cases (59 LGFMS, 52 non-LGFMS). Of all, 48/59 (sensitivity, 81%) LGFMS contained detectable transcripts (45 FUS-CREB3L2, 3 FUS-CREB3L1). Most relevant clinicopathologic features of fusion-positive LGFMS included predominance in lower extremities (22/48; thigh: 13/48), deep situation (46/48), and occasional presence of unusual histologic features, for example, hypercellular areas (16/48), foci of epithelioid cells (13/48), and giant rosettes (6/48). Most tumors expressed EMA (41/45), at least focally, CD99 (38/41) and bcl-2 (36/41) while being essentially negative for CD34 (2/45), mdm2 (1/41), smooth muscle actin (1/45), S100 protein (0/46), desmin (0/44), h-caldesmon (0/42), keratins (0/44), and CD117 (0/40). Eleven presumed LGFMS were fusion negative. Of all, 7/52 non-LGMFS neoplasms contained FUS-CREB3L2 transcripts, of which 4 had been diagnosed as sclerosing epithelioid fibrosarcoma. In conclusion, FUS-CREB3L1/L2 fusion transcripts can be detected in paraffin-embedded LGFMS in a sensitive manner, using reverse transcription-polymerase chain reaction. Most fusion-positive LGFMS are EMA-positive and CD34/S100/smooth muscle actin negative. The presence of epithelioid cells and fusion transcripts in both LGFMS and a subset of sclerosing epithelioid fibrosarcoma suggest that these neoplasms might be related.
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PMID:Translocation-positive low-grade fibromyxoid sarcoma: clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epithelioid fibrosarcoma: a study from the French Sarcoma Group. 1772 Nov 95

A 40-year-old Asian female presented with a 2-month history of right shoulder pain and right triceps weakness. MRI revealed an extramedullary, extradural, dumbbell-shaped spinal cord tumor with C6 to C7 iso- and hyperintensity on T1 and T2 weighted imaging, respectively. Histological examination revealed monomorphous spindle cells with a storiform pattern. Immunohistochemistry was positive for CD34, CD99, and negative for EMA, SMA, and S100; solitary fibrous tumor (SFT) was confirmed.
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PMID:A dumbbell-shaped solitary fibrous tumor of the cervical spinal cord. 1830 86

A 13 year old boy presented with a huge mass on his right arm of 6 months duration. Histopathological examination revealed sheets of malignant small round blue cells with immunopositivity for LCA, CD43, CD45Ro, CD30, EMA, ALK-1 and CD99, and negativity for CD20, TdT, myogenin, myoD1, NSE, bcl-6, bcl-2 and CD10. Fluorescent In-Situ Hybridization (FISH) testing excluded the diagnosis of Ewing's sarcoma/PNET. Pathologists need to be aware of the diagnosis of a small cell variant of ALCL, as well as of the fact that CD99 expression commonly occurs in cases of ALK-positive ALCL, in order to distinguish this entity from Ewing's sarcoma/PNET.
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PMID:Small cell variant of anaplastic large cell lymphoma with positive immunoreactivity for CD99. 1894 5

A case of adenoma of the non-pigmented ciliary epithelium with smooth muscle differentiation is reported. This uncommon ocular tumor affected a 36-year-old woman, and had caused decreased visual acuity and a total cataract. Ultrasound biomicroscopy disclosed an associated persistent hyperplasic primary vitreous (PHPV). Sectoral cyclectomy with removal of the mass and intracapsular cataract extraction were performed. The tumor was diffusely positive for vimentin, smooth muscle actin, NSE, and S-100, focally for CD68 and Melan-A, and was negative for desmin, EMA, HMB-45, and CD99. Occasional cells reacted for cytokeratin. The proliferation index, as assessed by Ki-67, was below 10%. The overlying non-neoplastic ciliary epithelium was positive for vimentin, NSE, and S-100. Myofilaments are not totally unexpected in ciliary adenomas; however, such a diffuse and strong positivity for smooth muscle actin, as in the present case, has only been observed in one case before, but should be considered in the differential diagnosis of these neoplasms.
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PMID:Adenoma of the non-pigmented ciliary epithelium: a rare intraocular tumor with unusual immunohistochemical findings. 1934 82

Superficial acral fibromyxoma (SAFM) is an uncommon tumor of the superficial soft tissues of acral sites. SAFM is a proliferation of fibroblastic cells, within a myxoid to collagenous stroma. The published cases mostly expressed immunoreactivity for CD34, CD99, EMA, and, less frequently, CD10. The authors report an additional case that did not express any of the previously reported markers, including CD34, and antigens of mesenchymal stromal lineage. Ultrastructural study confirmed the tumor cells were typical fibroblasts with cytoplasmic intermediate filaments and numerous cisternae of rough endoplasmic reticulum. The authors describe the first example of SAFM, ultrastructurally studied, with pure fibroblastic immunoprofile.
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PMID:Superficial acral fibromyxoma: immunohistochemical and ultrastructural analysis of a case, with literature review. 1992 77

A 68-year-old woman with stage pT1b1 N0 cervical squamous carcinoma had an incidental cervical polyp. The polyp measured 1.7 cm in maximal diameter and histologic evaluation showed it to be composed of spindle-shaped cells with hypercellular and hypocellular foci. The stroma was collagenized and contained several dilated vascular channels that imparted a hemangiopericytic pattern to the lesion. Immunohistochemistry showed the spindle cells to be positive for vimentin, CD99, CD34, bcl-2, ER, PR, and beta-catenin (cytoplasmic) but negative for EMA, S100, factor XIIIa, AE1/AE3, caldesmon, desmin, CD31, and smooth muscle actin. The morphology and immunophenotype was in keeping with a diagnosis of a solitary fibrous tumor (SFT). SFT shares several histologic features of a superficial cervicovaginal myofibroblastoma; the cellular variability, pattern and distribution of vessels, stromal collagenization, and desmin negativity favors SFT.
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PMID:Solitary fibrous tumor of the uterine cervix. 2017 6

Pure Sertoli cell tumor (SCT) is a rare sex cord tumor and a subtype of Sertoli-Leydig cell tumors according to the WHO Classification. They lack a Leydig cell component and do not contain the immature neoplastic stroma found in the neoplasms of the Sertoli-Leydig cell category. The age of the patients ranges between two and 79 years. Sertoli cell tumors occur in women of reproductive age but a few can also occur in children. The most common clinical presentation when occurring in children is isosexual pseudoprecocity. Women of reproductive age and postmenopausal women frequently present with abdominal pain, swelling and menstrual abnormalities. Occasionally SCTs occur in patients who have Peutz-Jeghers syndrome. The tumors are hormone functional in 40-60% of cases. They are often estrogenic, occasionally also androgenic or rarely both. Grossly they are usually yellow to brownish, solid or with several cystic areas. Microscopically they show always almost a tubular growth pattern, but they may also have other growth patterns which can be extensive, making the correct diagnosis difficult. These histologic patterns may result in SCTs mimicking other ovarian tumors. The immunohistochemical panel which usually includes EMA, inhibin, chromogranine, CD99 and calretinin is often helpful in establishing the diagnosis. Most SCTs are Stage I, unilateral, cytologically bland, and clinically benign, but occasional examples are high stage. About 11% of Stage I tumors have worrisome histologic features that may portend an adverse outcome.
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PMID:Pure Sertoli cell tumor. a case report and review of the literature. 2034 97

Solitary fibrous tumour (SFT) is a rare tumour principally found in adults in the pleural cavity. Extrapleural occurrences are rare. Two cases of SFT of the thyroid gland are described in this paper showing their distinctive microscopical architecture, namely "patternless growth pattern". It is characterized by a bland spindle-cell proliferation alternating hyper- and hypo-cellular areas, keloid-like hyalinization and a focal hemangiopericytoma-like vascular pattern. Tumour cells revealed a diffuse strong positivity for CD34, CD99, bcl-2 and Vimentin, but negativity for Desmin, EMA, AE1/AE3, SMA, S-100 and CD31 antibodies. The differential diagnosis of thyroid SFT includes different types of spindle cell proliferation, benign and malignant mesenchymal tumours, medullary thyroid carcinoma, fasciitis-like papillary carcinoma, and undifferentiated (anaplastic) carcinoma. However, the morphologic and immunohistochemical findings of SFT are so characteristic that this diagnosis seldom represent a difficulty.
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PMID:Solitary fibrous tumour of thyroid: report of two cases with immunohistochemical features and literature review. 2061 21

The first case of large cell neuroendocrine carcinoma arising in an infant is presented. The tumor arose at the anal verge of a 1-year-old girl. The diagnosis of this CD99-positive tumor was supported by expression of epithelial (keratins, EMA, and Ep-CAM) and neuroendocrine (chromogranin A, synaptophysin, and neuron-specific enolase) markers and absence of immunoreactivity for Fli-1. No fusion of EWSR1 with FLI-1 or ERG was detected by polymerase chain reaction. However, the split of the EWSR1 gene was demonstrated by fluorescence in situ hybridization. This case adds to the few epithelial tumors in which an EWSR1 rearrangement was demonstrated. Because the tumor was initially misclassified as an extraskeletal Ewing's sarcoma, the patient was treated according to the Ewing's sarcoma treatment protocol. She remains free of tumor 8 years after initial diagnosis.
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PMID:CD99-positive large cell neuroendocrine carcinoma with rearranged EWSR1 gene in an infant: a case of prognostically favorable tumor. 2061 39

A solitary fibrous tumor of the pleura (SFTP) is an uncommon tumor, in contrast to a relatively common mesothelioma in pleura. Its average size varies from 6 to 8 cm. We report herein a case of a giant SFTP in a 53-year-old man who presented with cough since five years along with chest pain on the left side and progressive dyspnea since two months. Radiological findings revealed a large pleural mass measuring 25 cm in its largest dimension, filling the pleural cavity with effusion. Biopsy showed a spindle cell tumor with areas of dense sclerosis. Subsequent excision unraveled a large multinodular, grey-white tumor, histologically, composed of spindle cells in a 'patternless' arrangement in dense collagenous stroma with areas of hyalinization. Focal areas showed hypercellularity with atypia and mitoses, but less than 4/10 High-Power Field, unassociated with necrosis. On immunohistochemistry, tumor cells showed diffuse positivity with vimentin, CD34 and BCL2 along with cytoplasmic positivity for MIC2 (CD99), whereas cytokeratin, EMA, calretinin and HBME-1 were negative. Diagnosis of an SFTP was substantiated over a close differential of a desmoplastic mesothelioma. In view of atypical features, a close follow-up of the case was recommended.
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PMID:A giant solitary fibrous tumor of the pleura: diagnostic implications in an unusual case with literature review. 2069 22


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