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

Proximal type epithelioid sarcoma is a rare neoplasia in which morphological findings are characterized by nodular proliferation of epithelioid cells with focal rhabdoid features. It shares some histological features with other neoplasias and this gives an account of several differential diagnosis with other extrarenal rhabdoid tumors. Immunohistochemical and ultrastructural analysis are important in defining this entity: vimentin, cytokeratin, EMA and often CD34 expression of tumoral cells, moreover ultrastructurally evidence of large paranuclear whorls of intermediate filaments, are requested for diagnosis. A correct diagnostic framing is necessary because of the aggressive clinical behaviour of this tumor, that has a tendency to early spreading. We describe a case of vulvar proximal type epithelioid sarcoma in a 34 years old woman.
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PMID:["Proximal type" epithelioid sarcoma of the vulva: differential diagnosis with other extrarenal rhabdoid tumors]. 1625 80

To investigate the clinicopathological characteristics and biologic behavior of epithelioid hemangioma in oral mucosa, the clinical features and pathological findings of seven cases of epithelioid hemangioma were reviewed, and all sections were examined by immunohistochemistry. There were 4 males and 3 females ranged from 8 to 65 years (mean 39.7 years). Sites of origin included the tongue (n = 4), lips (n = 2), and palates (n = 1). Five lesions presented as a solitary mass. Three patients had history of minimal trauma. Histopathologically, well-formed but often immature vessels lined by plump epitholid endothelial cells with prominent inflammatory components were observed. Immunohistochemical staining revealed that tumour cells were positive for Vimentin, FVIIIRAg and CD34. All the cases were negative to S-100 protein and EMA. One case recurred after surgical excision, while none of them metastasized. Epithelioid hemangioma is a benign vascular anomaly, and has close relationship with local trauma. The differential diagnosis is usually based upon histopathological findings.
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PMID:Epithelioid hemangioma in the oral mucosa: a clinicopathological study of seven cases and review of the literature. 1626 21

An 83-year-old woman was admitted to our hospital because of colicky pain under the right costal arch suggesting cholecystitis. Physical examination confirmed by ultrasound scan indicated a palpable tumor in that location. Laparotomy was performed and the tumor was removed. Histopathological examination revealed malignant peripheral nerve sheath tumor (MPNST) originating in neurofibroma of the mesentery. Macroscopic, histological and cytological features were typical for MPNST. High nuclear pleomorphism, hyperchromasia were observed but on average only two mitotic figures per high power field were seen. The growth fraction determined by Ki-67 immunoreactivity was about 30%. Immunohistochemical stains revealed positivity of individual cells for NK-1(CD57), S-100 protein and NSE. It was lack of positivity for cytokeratin, EMA, vimentin, desmin, SMA, CD34. We report a well documented case of MPNST originating in preexisting neurofibroma of the mesentery. To our knowledge, is the first case in the Polish literature.
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PMID:Malignant peripheral nerve sheath tumor originating in neurofibroma of the mesentery. Case report. 1633 83

We report five cases of sporadic meningioangiomatosis, three males and two females, ranging in age from 12 to 36 years at diagnosis. The lesion was found incidentally by MRI after a head trauma in one case; the other four subjects had a seizure disorders, which improved following surgical resection of the cortical lesions. Grossly, the lesionectomy specimens were of a whitish color and firm consistency. Histological examination revealed that the lesions were confined to the cortex with focal involvement of the overlying leptomeninges, and revealed unifying features of meningioangiomatosis, such as proliferating microvessels with perivascular cuffs of spindle-cell proliferation within the cortex. Two cases had numerous calcifications; one was associated with a prominent fibrocalcifying component. Immunostaining results were variable among the cases. Only vimentin was consistently positive. Some of the spindle cells were weak positive for EMA in two cases. Immunoreactions with anti-CD34 detected within the cytoplasm of the spindle cells were observed in three of the five cases. The Ki-67 proliferation index of all the cases was very low, less than 0.1%. Neurofibrillary tangles were identified in only one of the five cases using the Bodian and immunostaining methods. These findings indicate that meningioangiomatosis lesions show a wide range of clinicopathological features, making diagnosis difficult. A histopathological spectrum and differential diagnoses were discussed with a review of the literature. Since this lesion is a distinct clinicopathological entity and hamartomatous in nature, it is important to make a correct diagnosis in order to avoid further aggressive treatment.
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PMID:Histopathological study of five cases with sporadic meningioangiomatosis. 1677 Nov 83

Haemangiopericytomas originate in extravascular cells (pericytes). They are well-differentiated tumours with low potential for local recurrence or metastasis. The most common presenting symptoms are nasal obstruction and recurrent epistaxis. Differential diagnosis requires immunohistochemical staining. Treatment consists in wide surgical excision. Case report a 44-years old male turned up with symptoms of right-sided nasal obstruction and epistaxis. A reddish, tough tumour 10 mm in diameter was diagnosed, covered with unchanged mucous membrane with small ulceration 3 mm in diameter, growing in the nasal septum. The tumour was dissected from unchanged septal cartilage and removed under endoscopic guidance. Histopathological examination disclosed prolonged, multiangular cells, with no mitotic activity. Focal faint expression of actin was confirmed, while the tumour cells were negative for CD34, CD31, GFAP, EMA, CK and S-100. Endothelial cells were positive for CD31 and CD34. Epithelial cells were positive for EMA and CK. The results of differential diagnosis were consistent with the diagnosis of sinonasal haemongiopericytoma. The patient has been asymptomatic for 6 months. Conclusion. Transnasal endoscopic approach to the discussed sinonasal haemangiopericytoma located on the nasal septum allowed its optimal visualisation and removal with macroscopically sufficient margins.
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PMID:[Sinonasal haemangiopericytoma of the nasal septum removed under endoscopic guidance]. 1688 73

Leiomyomatosis peritonealis disseminata (LPD) is a rare disease presenting as multiple peritoneal nodules of smooth muscle cells, mimicking peritoneal carcinomatosis. This disease usually pursues a benign course. We report one case of LPD in a 32-year-old woman, G2P1, without gynecological history. At term, she had an elective caesarean section during which several firm peritoneal nodules, ranging from 0.2 to 0.4 mm, were found. Microscopic examination showed a smooth-muscle cell proliferation with no mitosis, no atypia and no necrosis. Immunohistochemical analysis showed diffuse and strong staining for progesterone receptors and partial expression of estrogen receptors. The cells coexpressed smooth muscle actin, desmin, h-caldesmon, calretinin, WT1, and CD117 (KIT). They were weakly positive for EMA but negative for CD34. Proliferation index was low with 5% of cells being positive for MIB-1. This case of LPD proved to have an unusual immunohistochemistry profile raising the question of its real origin.
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PMID:[Leiomyomatosis peritonealis disseminata: immunohistochemical profile and origin]. 1712 52

Clear cell sarcoma (CCS) is a high grade soft tissue sarcoma with a distinct molecular profile and with morphological features resembling those of melanoma. CCS has been rarely described in other locations other than the soft tissues, including the gastrointestinal tract. In this study, we report a case of CCS arising in the ileum of a 31-year-old woman. Histologically, the tumor involved the entire thickness of the intestinal wall. Tumor cells were polygonal or fusiform, with clear or eosinophilic cytoplasm, arranged in a uniform nested to fascicular growth pattern. Immunohistochemical studies revealed strong positivity for vimentin and S-100 protein. HMB-45, Melan-A, tyrosinase, cytokeratins, EMA, smooth muscle actin, CD34, CD31, CD117, CD99, synaptophysin, chromogranin A, CD56, and NSE were negative. Fluorescence in situ hybridization analysis demonstrated the presence of a t(12;22)(q13;q12) translocation, the diagnostic hallmark of CCS of soft parts. The present case, together with a detailed review of the literature on this topic, demonstrates that the gastrointestinal tract is a possible site of CCS of soft tissues and that making a reliable diagnosis of this tumor requires cytogenetic or molecular diagnostic investigations.
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PMID:Clear cell sarcoma of the ileum: report of a case and review of literature. 1763 26

Computed tomography revealed a well-enhanced omental mass. Magnetic resonance imaging demonstrated a mass with low signal intensity on T1-weighted images (WI) and high signal intensity on T2-WI. Resected specimens immunohistochemically showed positive results for alpha-smooth muscle actin, muscle-specific actin (HHF35) and vimentin, and negative results for S-100 protein, CD34, desmin, EMA, keratin, calretinin, HBME1, and c-kit. This is the first case of an omental glomus tumor reported in the English literature.
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PMID:Glomus tumor of the omentum: a case report. 1770 47

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

We report 3 patients with fibrous solitary tumor of meningeal location where we described the histological study, as well as evolution after the surgical treatment. The described patients presented ages of 37, 52 and 65 years, after the resection has not appeared an objective sign of recurrence in any case after 4, 6 and 7 years of follow-up respectively. Checking the literature the tumor is indistinguishable clinical and radiolocally of the typical meningioma, doing necessary the use of inmunohistochemistry to do the differential diagnosis, where positiveness for CD34 and the negativeness for EMA define the fibrous solitary tumor. It is about a benign tumor, where total removing is the principal factor in prognosis, nevertheless there are cases of local recurrences and long-distance metastasis. We can find all these characteristics in the showed cases of the present article, having the uncertainty of its local or systemic relapse ability in the future.
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PMID:[Solitary fibrous tumors of the meninges: report of three cases and literature review]. 1809 9


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