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

Analogous to mixed tumors of salivary glands (" pleomorphic adenomas" ), cutaneous mixed tumors (" chondroid syringomas" ) contain a ductal (epithelial) component and a variably prominent myoepithelial component. Tumors showing purely myoepithelial differentiation (myoepitheliomas) have only recently been recognized to arise in the dermis, and to date very few cases have been described. To characterize these tumors further, 14 cutaneous myoepithelial tumors were retrieved from the authors' consult files. Eleven patients were male and 3 were female; their median age was 22.5 years (range, 10 to 63 years), and 7 patients were between 10 and 20 years old. Tumor size ranged from 0.5 to 2.5 cm (mean, 1.1 cm). Most tumors arose on the extremities: 6 on the upper limbs, 6 on the lower limbs, and 1 each on the back and nose. Ten tumors were limited to the dermis, and 5 also extended into superficial subcutis. Thirteen tumors were myoepitheliomas (lacking ductal differentiation), and 1 tumor was a myoepithelial carcinoma (exhibiting severe cytological atypia and a high mitotic rate). Histologically, 7 tumors were solid, composed of ovoid to spindled, histiocytoid, or epithelioid cells with no significant stroma, and 7 were predominantly lobulated, with cords or nests of epithelioid, plasmacytoid, or spindled cells with a variably reticular architecture and a chondromyxoid or collagenous/hyalinized stroma. One tumor was composed solely of plasmacytoid (hyaline) cells, and 1 exhibited extensive adipocytic differentiation. Among the 13 myoepitheliomas, mitoses ranged from 0 to 6 per 10 high-power fields (HPFs) (mean, 1.5); 8 tumors contained no mitoses. The myoepithelial carcinoma had 39 mitoses per 10 HPFs. By immunohistochemistry, all cases were reactive for epithelial markers (keratins and/or epithelial membrane antigen [EMA]); 13 of 14 (93%) expressed S-100 protein, 10 of 11 expressed (91%) calponin, 11 of 14 (79%) expressed EMA, 9 of 14 (64%) expressed keratins, 8 of 14 (57%) expressed smooth muscle actin, 7 of 14 (50%) expressed glial fibrillary acidic protein, 3 of 11 (27%) expressed p63, and 1 of 6 (17%) expressed desmin. All 5 cases without keratin staining were diffusely positive for EMA, and all of these cases showed a solid growth pattern. Follow-up was available for 8 patients (median follow-up, 40 months; range, 6 months to 9 years); 3 tumors (38%) recurred locally, and 1 tumor (13%) also metastasized to the lymph nodes. The case that resulted in recurrence and metastasis had the highest mitotic rate (6 per 10 HPFs) of the cytologically benign tumors. Follow-up information was not available for the myoepithelial carcinoma. This study suggests that approximately 50% of cutaneous myoepitheliomas are distinctive lesions composed of a solid proliferation of cells with abundant eosinophilic syncytial cytoplasm, which often lack immunostaining for keratin, whereas the remainder demonstrate focally reticular architecture and myxoid stroma or plasmacytoid cells, similar to their counterparts in salivary gland and soft tissue. Whereas most cutaneous myoepitheliomas behave in a benign fashion, there is apparently a significant risk for local recurrence but a low metastatic potential.
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PMID:Cutaneous myoepithelioma: a clinicopathologic and immunohistochemical study of 14 cases. 1474 20

Extracranial spread of neuroectodermal tumors is an unusual event, most frequently expected from glioblastomas and medulloblastomas. Single cases of metastatic oligodendrogliomas have been described, but no genetic data are reported. Oligodendrogliomas are characterized by distinct genetic alterations, i.e. loss of heterozygosity (LOH) of 1p and 19q; therefore, molecular genetic analysis of metastatic cases is of considerable interest. It may be instrumental in defining the distant tumor as metastatic oligodendroglioma and give clues to the genetic events associated with the highly malignant transformation. We present the case of a patient with multiple bone metastases from a cerebral oligodendroglioma. Oligodendroglioma grade II was the diagnosis both at original and second operation, performed 7 and 1 years before the extracranial dissemination. The extraneural spread presented before the local intracranial recurrence. The patient received procarbazine, lomustine, vincristine chemotherapy and radiotherapy after the second surgery. The computed tomography-guided biopsy of the bone lesions revealed tumor cells positive for GFAP, S-100 and Leu-7 and negative for cytokeratin, LCA and EMA. The genetic analysis of DNA from the original tumor, the bone metastasis and the autoptic brain tumor showed LOH of 1p; heterozygous deletion of CDKN2A/p 16 was detected as additional alteration in the metastasis and in the intracranial tumor at autopsy. TP53, MDM2 and CDKN2A/p14ARF genes were unchanged. Repeated brain surgery and extended survival may have acted as promoter of extraneural dissemination. Loss of CDKN2A most probably played an important role in the malignant progression: its involvement in metastatic potential remains to be clarified. Our data confirm that malignant transformation of oliogodendrogliomas may be undetected by histology and underscore the importance of genetic analysis. Coincidentally with intensive anticancer therapy, chemotherapy included, employed in patients with oligodendroglioma and the ensuing long survival, the frequency of metastatic oliogodendrogliomas may increase.
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PMID:Molecular genetic study of a metastatic oligodendroglioma. 1501 56

Multidirectional differentiation of neoplastic cells in uterine MMT is still a subject of controversy. The present study was designed to assess the immunophenotype of 15 uterine MMT paying special attention to the markers of neural (neuroendocrine) differentiation. In addition, the same immunohistochemical study was performed on 20 human fetal specimens in order to establish possible relationships between the immunophenotype of MMT and the expression of the corresponding antigens in the fetal tissues of the embryonal female genital tract. Besides the typical immunohistochemical patterns in three cases the epithelial component showed simultaneous coexpression of vimentin and desmin. EMA and cytokeratin, whereas epithelial markers were coexpressed with vimentin in the sarcomatous component of one adenosarcoma. Moreover, both components were immunoreactive to the markers of neural differentiation (PGP 9.5, GFAP, HNK-1, N-CAM, HBA71). This aberrant expression was not correlated with morphological signs of neural differentiation at either light microscopy or ultrastructural levels. Regarding the analysis of fetal tissues, both epithelial and mesenchymal elements in the fetal genital tract expressed the above-mentioned neural markers at different dates of gestation. The intensity of this expression diminishes as the fetus matures and at the end of antenatal life the immunophenotype characteristic for adult life is established. Taking into consideration the capacity of uterine tissue to reproduce embryonal phenotype during neoplastic transformation, we studied this abnormal immunoprofile and its hypothetic value for the diagnosis and prognosis of MMT.
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PMID:Neuroectodermal immunophenotype in uterine malignant mullerian tumors (MMT): comparative immunohistochemical analysis with embryonal uterine development. 1505 66

Ectomesenchymal chondromyxoid tumour (ECT) of the anterior tongue was first described in 1995. To date, only 23 cases have been reported in the literature. Two new cases of ECT have been described, with immunohistochemical analysis including cytokeratin (CK) profile, GFAP, S-100 protein, SMA, CD-57, EMA, desmin and Ki67. Tumour cells showed intense and diffuse staining for GFAP and diffuse staining for S-100 protein. Pan-keratin, high- and low-molecular-weight CK, CK 7, 8, 18, 19 and 20 were negative. Tumour cells were also negative for desmin, SMA, CD-57 and EMA. Ki67 was positive in only scattered cells. The findings of the present study support the suggested ectomesenchymal origin for ECT, rather than myoepithelial salivary gland origin. The low Ki67 expression is in agreement with the low growth rate, small size and lack of mitotic activity in the present cases, as well as in those previously described.
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PMID:Ectomesenchymal chondromyxoid tumour of the anterior tongue. 1514 46

A case of capillary hemangioblastoma located in the peripheral soft tissue of the inner ankle in a 74-year-old woman is presented. The tumor was an unencapsulated but sharply circumscribed nodule 2.5 cm in size, of a yellow-white color. It showed reddish-brown spots with small cysts up to 2 mm filled with blood. Grossly the tumor was not attached to any peripheral nerve. Signs of von Hippel-Lindau's disease were excluded by thorough clinical evaluation. No additional tumor or erythrocytosis was found in the patient clinically. Immunohistochemically, the tumor stromal cell reacted strongly with antibodies to S-100 protein, NSE, and calponin and they were negative with antibodies to GFAP, CD34, CD31, cytokeratins, actin, desmin, EMA, and HMB-45. Endothelium of the capillaries reacted positively with antibodies to CD31, CD34, and Factor VIII-related protein. Capillary pericytes were actin-positive. All cells of the tumor stained positively with antibody to vimentin. MIB1 antibody reacted only in very few cells (<1%). Ultrastructurally, the stromal cells contained electron-lucent cytoplasm with lipid droplets, a small amount of rough endoplasmic reticulum, and glycogen particles. No electron-dense structures typical of secretory granules were seen in the stromal cells. No mutation of coding sequence of VHL gene was found.
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PMID:Primary capillary hemangioblastoma of peripheral soft tissues. 1522 69

Ectopic adrenal cortical neoplasms are extremely rare, and only a few have involved the CNS. We report the first case of an intramedullary oncocytic adrenal cortical neoplasm of the spinal cord with immunohistochemical (IMHC) confirmation. A 27-year-old man presented with progressive lower extremity weakness, spastic paraparesis, decreased reflexes, and hypoesthesia below T10. A spinal myelogram showed cauda equina blockade and obliteration of sacral nerve roots. This prompted emergent surgical intervention. A well-circumscribed, approximately 3 x 2 cm, light brown to tan, intramedullary tumor was identified at the level of the conus medullaris. Histologically, the tumor showed sheets and nests of plump, cytologically bland polygonal cells with abundant eosinophilic cytoplasm. A single mitosis, but no necrosis, was identified. By IMHC, the cells were positive for inhibin, melan-A, and synaptophysin, and negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin. Electron microscopy study performed from paraffin-embedded tissues demonstrated abundant mitochondria, and lipid vacuoles. This case confirms the occurrence of adrenal cortical neoplasms in the CNS and is the first report of an intradural, intramedullary adrenal cortical adenoma of the spinal cord, and the first to occur in a male. This tumor should be considered in the differential diagnosis of tumors of the CNS.
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PMID:Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature. 1530 40

Rare examples of high-grade gliomas show focal epithelial differentiation, which may require distinction from colliision tumors. These epithelial constituents are not well-characterized immunophenotypically and have rarely been subjected to genotyping. We describe a case of a 54-year-old female with a short history of hemiparesis who was found to have an absolute lymphocytosis and a heterogeneously enhancing frontotemporal tumor. Cytological and histological examination of brain biopsies confirmed the presence of a glioblastoma multiforme also containing CAM5.2/CK7/BerEP4/CEA/EMA-immunopositive and GFAP-immunonegative nests of epithelial cells with a high proliferative index and focal glandular differentiation. Hematological investigations confirmed a diagn of chronic lymphocytic leukemia (CLL) with no demonstrable CNS involvement. Genetic analysis using microsatellite markers and specimens obtained by laser capture microdissection of the CNS tumor and normal brain tissue, showed that both the glial and epithelial components of the brain tumor had identical losses of 2/2 informative markers on chromosome 17p13 and 5/5 informative markers on chromosome 10q22-26. The glial and epithelial components also shared an identical 2 base pair deletion in the TP53 gene at codon 209, exon 6, introducing a stop codon at codon 214. No losses at any of the above loci and no TP53 mutation were detected in the leukemic cells. These results suggest both components of the brain tumor, although differing in phenotype, share the same genetic lineage.
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PMID:Phenotypic and genotypic characterization of glioblastoma multiforme with epithelial differentiation and adenoid formations. 1532 77

The spindle cell variant of rhabdomyosarcoma (RMS) is uncommon and is most often encountered in the paratesticular region of children in whom it has a good prognosis. Only isolated cases in adulthood have been described. Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. Eleven patients were male and 5 were female. Patient age ranged from 18 to 79 years (median, 32 years). Tumor size varied from 1.5 to 35 cm (median, 6 cm). The head and neck region, including the oral cavity, parotid gland, nasopharynx, and nasal cavity, was the commonest affected area, accounting for >50% of the cases, followed by retroperitoneum, thigh, leg, subscapular area, hand, vulva, and paratesticular region (1 case each). Follow-up was available in 12 cases, ranging from 1 to 102 months (median, 16.5 months). Treatment modalities included surgery, chemotherapy, and radiation. Two patients died of uncontrolled local disease 13 and 27 months after diagnosis; 4 were alive without disease at 12, 17, 24, and 102 months, including 1 patient with metastasis to 10 of 50 pelvic lymph nodes at presentation; 3 are alive with localized disease at 16, 17, and 19 months; and 1 was followed for 6 months and showed persistent local disease. One patient is alive at 10 months after diagnosis with evidence of metastatic disease to bone, lungs, and breast. All the tumors showed long fascicles of spindle cells with elongated, vesicular nuclei and pale indistinct cytoplasm. Scattered spindled or polygonal rhabdomyoblasts with abundant brightly eosinophilic cytoplasm were present in all cases. In 3 cases, focal areas showed pseudovascular, sclerosing features. There were no round cell or pleomorphic areas. Positive immunohistochemical results were as follows: desmin (15 of 15 cases), myf-4 (12 of 12), fast myosin (7 of 9), myoglobin (2 of 3), HHF-35 (9 of 9), and SMA (11 of 14). One tumor was focally positive for keratins and EMA. All tumors were negative for caldesmon, S-100 protein, and GFAP. Spindle cell RMS is a rare neoplasm in adults and appears to have distinct clinicopathologic features when compared with cases occurring in the pediatric population. Specifically, it appears to be most common in the head and neck region, and although only limited follow-up is available so far, these lesions appear to have a more aggressive clinical course in adults.
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PMID:Spindle cell rhabdomyosarcoma in adults. 1600 7

The case of a soft tissue myoepithelioma is presented including clinicopathologic, ultrastructural, and genetic findings. A 30-year-old male patient suffered from a soft tissue tumor within the deep soft tissues of the right lower leg measuring 13.2 x 8.2 x 9 cm. Histologically, the lesion was diagnosed as a myoepithelioma displaying a lobulated architecture with cords and nests of epithelioid and spindle cells without cytologic atypia lying within a fibromyxoid and partly chondroid matrix; immunohistochemistry was positive for pancytokeratin, S100-protein, calponin and partly for GFAP and EMA. Ultrastructural analysis revealed glycogen deposits and cell-membrane-associated plaque structures, whereas true myofilaments could not be identified (with immunohistochemistry being negative for actin). Using comparative genomic hybridization (CGH), a gain of chromosome Y was detected. A loss on 17p could not be detected unambiguously. However, based on the low resolution of CGH a small loss cannot be excluded. The patient was free of disease 25 months following complete tumor resection. Myoepitheliomas/mixed tumors of deep soft tissue represent rare soft tissue lesions that may reach a considerable size and may mimic other soft tissue tumors or sarcomas. Based on a local relapse rate of approximately 20% according to the literature, a complete resection with thorough follow-up should be recommended.
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PMID:[Myoepithelioma of soft tissue -- case report with clinicopathologic, ultrastructural, and cytogenetic findings]. 1603 88

We report a primary retinal tumor with features of myxopapillary ependymoma. The lesion occurred in a 33-year-old man with a long history of phthisis bulbi and a more recent history of pain to the right eye. Enucleated ocular globe revealed a lesion occupying most of the retinal surface. Histologically, the retina was replaced by a tumor composed of spindle cells with fibrillary cytoplasm and round to ovoid nuclei forming fascicles, perivascular pseudorosettes, microcysts, and deposition of extracellular mucins. Calcifications, metaplastic bone, and lymphoplasmacytic inflammatory infiltrate were also seen. Tumor cells expressed GFAP and S-100 and to lesser extent carbonic anhydrase II. The immunoreaction for EMA showed diffuse granular positivity, decorated a few extracellular lumina, and highlighted intracytoplasmic lumina in a few cells. Ultrastructurally, there was accumulation of extracellular material between cells and around capillaries, long interdigitating cytoplasmic processes, extracellular lumina packed with microvilli, a few junctions evident around lumina, and some ciliary basal bodies and ciliary basal rootlets. As control cases, we also investigated expression of EMA and carbonic anhydrase II in an ocular globe with retinal gliosis and three cases of myxopapillary ependymoma of the cauda equina. The lesion described here represents the first example of retinal tumor with features of myxopapillary ependymoma. Pathologic features and particularly expression of carbonic anhydrase II suggest a derivation from intrinsic glial cells of retina otherwise known as Muller cells.
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PMID:Primary glial tumor of the retina with features of myxopapillary ependymoma. 1616 Apr 86


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