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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

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

We discuss here five cases of epithelioid sarcoma (ES) with final diagnosis established after reexamination of initial findings. Problems with differential diagnosis of these neoplasms arise since their microscopic picture may simulate several other pathological conditions such as non-neoplastic granulomatous reactions, squamous cell carcinomas and adenocarcinomas, melanomas and soft tissue sarcomas with epithelioid component. Final ES diagnosis requires presence of cytokeratin, EMA and vimentin in neoplastic cells, as confirmed by immunohistochemical reactions. Differential diagnosis is also helped by concurrent cytology assessment that allows recognizing more easily such characteristic features as presence of plasmacytoid or spindle-shaped cells.
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PMID:Diagnostic problems concerning epithelioid sarcoma--case analysis. 1633 82

A case of spinal thoracic chordoma involving the T9 vertebra in a 70-year-old male patient, destroying the vertebral body and invading the vertebral canal with compression of the spinal cord, is presented. The patient was referred to our neurosurgical unit with a history of an irradiated metastatic adenocarcinoma to the thoracic vertebra, a diagnosis that was rendered 3 years earlier at another hospital on presentation. This misdiagnosis was likely due to the absolute rarity of thoracic vertebral chordomas (2%-3% of all chordomas), the higher frequency of metastatic deposits to the vertebrae from visceral cancers in the elderly, the limited amount of biopsy material available for histologic examination, and the epithelial phenotype of the tumor (keratin/EMA positive). The patient underwent second palliative surgery with subtotal piecemeal removal of the tumor bringing relief of the neurologic symptoms. The bulk of the tumor was represented by a high-grade pleomorphic sarcoma with adjacent areas of atypical chordoma. Small foci of conventional chordoma were also found. The previous histologic slides were also reviewed, which were consistent with the areas of atypical chordoma. Small targeted tissue fragments from areas of (atypical) chordoma and from sarcomatous areas were recovered for electron microscopy. The fine features of chordoma and focal rhabdomyoblastic differentiation were found with the latter retrospectively supported by immunohistochemical detection of striated muscle markers. A final diagnosis of dedifferentiated chordoma with rhabdomyoblastic differentiation was finally established. Rhabdomyoblastic metaplasia is a novelty in dedifferentiated chordoma. The patient died after 5 months. Autopsy was not requested.
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PMID:Dedifferentiated chordoma of the thoracic spine with rhabdomyosarcomatous differentiation. Report of a case and review of the literature. 1763 Jan 10

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

We report 2 cases of Follicular Dendritic Cell Sarcomas of Oral Cavity in two elderly patients. The patients presented with oral cavity tumors. Initial tru-cut biopsies in both cases revealed spindle cell neoplasms. One of them was of low grade malignancy and showed positivity for of Vimentin. Few cells showed positivity for keratin and were negative for S-100 protein, CD 34, CD 68, EMA, SMA, HMB-45. A possible diagnosis of low grade fibrohistiocytic tumor was made. He underwent Lt total maxillectomy. Three years later he presented back with regional nodal metastasis. The regional lymph nodes showed features of follicular dendritic cell sarcoma. The second case revealed high grade spindle cell neoplasm and showed positivity for vimentin and S100 protein and was negative for EMA, keratin, CD-34, desmin, muscle actin and HMB-45. He was offered initial radio-therapy followed by hemifacial resection. The histology along with immuno histochemistry favoured a diagnosis of follicular dendritic cell sarcoma. He presented with local recurrence two months later. Both the cases are discussed in detail.
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PMID:Follicular dendritic cell sarcoma of oral cavity: report of 2 cases. 1788 29

Acral myxoinflammatory fibroblastic sarcoma (AMIFS) is a low-grade sarcoma that presents mostly in distal extremities of middle-aged patients. The clinicopathologic features, immunohistochemical profile and follow-up data of five cases (three men and two women; age 39-65 years) are presented. The tumors presented as a slow-growing, poorly circumscribed, subcutaneous masses in the hands (three), foot (one) and calf (one), with dermal involvement in two cases. They had myxoid and hyaline stroma with dense acute and chronic inflammation. Spindle cells, large bizarre ganglion-like cells and multivacuolated cells were seen. Variable reactivity in lesional cells were noted for vimentin, Alpha-1-antitrypsin (A1AT), factor XIIIa, CD68, CD95, CD117, Alpha-1-antichymotrypsin (A1ACT), CD34, AE1/3, S-100 protein, EBER, CD63 and CD15. MIB-1 showed 5-30% nuclear labeling. They were negative for cytokeratin AE1/3, smooth muscle actin, CD30, ALK-1, EMA, desmin, CMV, HMB-45 and Melan-A. Follow up ranged from 2 weeks to 95 months (mean 54). One patient was lost to follow up; three underwent excision and one patient had below the knee amputation. Two patients developed metastases (one died of disease), and two patients are alive without evidence of disease. AMIFS are rare tumors that may involve joints and tendons leading to clinical diagnosis of ganglion cyst or tenosynovitis.
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PMID:Acral myxoinflammatory fibroblastic sarcomas: are they all low-grade neoplasms? 1819 Apr 43

We report 2 patients with conventional prostatic adenocarcinoma who developed sarcomatoid carcinoma of probable prostatic origin 6 and 2.5 years after radiation treatment (seed implantation and external beam). Our cases had histologic features consistent with those cases previously reported in the literature. The tumors consisted of spindle cells with large hyperchromatic nuclei and a pattern mimicking a sarcoma. Immunohistochemical studies showed the tumors to be weakly positive for EMA, CK7, and vimentin. Ki67 staining showed positivity in more than 50% of tumor cells. The tumors also stained diffusely positive for p53 and p63. PSA and PAP were negative. Clinically, the sarcomatoid carcinomas appeared to be of prostatic origin. The pathogenesis of the tumors is still uncertain but most likely represent a radiation-induced dedifferentiation of prostatic adenocarcinoma.
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PMID:Sarcomatoid carcinoma after radiation treatment of prostatic adenocarcinoma. 1832 77

We report a case of an undifferentiated pancreatic carcinoma with osteoclast-like giant cells with focal osteochondroid differentiation in a 66-year-old man, who presented with painless jaundice, pruritus, and weight loss. Imaging studies revealed an inhomogeneous mass in the head of the pancreas. A pylorus preserving pancreaticoduodenectomy was performed. The resection specimen revealed a 9.5 x 4.2 x 3.2 cm(3) solid neoplasm in the pancreatic head with direct extension into duodenum and common bile duct. Microscopy showed a cellular neoplasm composed of pleomorphic mononuclear cells (pancytokeratin, and EMA-positive; LCA, and CD68 negative) and osteoclast-like multinucleated giant cells (vimentin, LCA, and CD68-positive; pancytokeratin, and EMA-negative) consistent with OGTP. The tumor contained a focal area of osteochondroid differentiation. Evidence supports that the tumor giant cells are non-neoplastic and of histiocytic origin. Osteochondroid differentiation within undifferentiated carcinoma is unusual; its presence might suggest a sarcoma diagnosis on biopsy material.
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PMID:Undifferentiated pancreatic carcinoma with osteoclast-like giant cells: report of a case with osteochondroid differentiation. 1914 1

By analogy to gliosarcoma, the term "ependymosarcoma" has recently been coined to thematize the rare phenomenon of a malignant mesenchymal component arising within an ependymoma. We report on an example of this paradigm, involving tanycytic ependymoma as the host tumor in a 40-year-old female who underwent two tumor extirpation procedures at one-year interval. She first presented with severe headaches, and was seen by imaging to harbor a moderately enhancing mass 2.5cm in diameter at the rostral septum pellucidum accompanied by occlusive hydrocephalus. Microscopically, the tumor consisted of solid, wavy fascicles of elongated cells that were occasionally interrupted by vague perivascular pseudorosettes. Mitotic activity was absent, and less than 1% of nuclei immunoreacted for MIB-1. A histological diagnosis of tanycytic ependymoma (WHO grade II) was rendered, and no adjuvant therapy given. At recurrence, the lesion was 3.5cm in diameter, intensely enhancing, and had already seeded into the subarachnoid space. Histology showed a biphasic glial-sarcomatous architecture with remnants of the original ependymoma now displaying hypercellularity and atypical - yet not frankly anaplastic - features. The sarcomatous moiety consisted of spindle and epithelioid cells densely interwoven with reticulin fibers. While the ependymal component was GFAP and S100 protein positive, and featured punctate staining for EMA, none of these markers was expressed in the adjacent sarcoma. Instead, the latter reacted for vimentin and smooth muscle actin. To the best of our knowledge, this is the first documentation of tanycytic ependymoma undergoing malignant transformation, one driven by a highly anaplastic mesenchymal component, corresponding to "ependymosarcoma".
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PMID:Rapid spontaneous malignant progression of supratentorial tanycytic ependymoma with sarcomatous features - "Ependymosarcoma". 1985 84


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