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)

Sarcomatoid carcinomas involving the intestinal tract are rare and usually associated with poor prognosis. We report a case of sarcomatoid carcinoma of the ileum, diagnosed in a 61-year-old man. The patient presented with acute intestinal occlusion. Surgical resection of the ileum was performed. At macroscopic examination, two large polypoid masses were found. Frozen section examination suggested the diagnosis of malignant stromal tumor. At histological examination, both tumors were formed by pleiomorphic, large spindle cells, presenting numerous mitoses and marked nuclear atypia. Immunohistochemical examination showed that tumor cells coexpressed vimentin and epithelial markers (cytokeratins, EMA). The final diagnosis was monomorphic sarcomatoid carcinoma. The patient deceased with metastatic disease after 3 months of follow-up. This report underlines the potential diagnostic problems raised by this unusual type of carcinoma and emphasizes the role of immunohistochemistry in assessing the correct diagnosis.
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PMID:[Sarcomatoid carcinoma of small intestine: diagnostic problems raised by an unusual tumor]. 1193

Immunohistochemical studies on metastatic carcinomas of unknown primary site are cost-effective and often allow a specific identification of the tumour origin, especially if the metastases are adenocarcinomas by light microscopy. Commercially available site-specific markers include prostate-specific antigen, thyroglobulin, thyroid transcription factor-1, uroplakin III, GCDFP-15, oestrogen and progesterone receptors, alpha-fetoprotein, the A103 monoclonal antibody against MART-1, cytokeratins 7 and 20, cytokeratins of basal cell type, p63, carcinoembryonic antigen, CA125, EMA, vimentin, HepPar-1, WT-1 and S100 protein. However, immunostaining with most of these markers does not show an absolute specificity for a certain primary site. For this reason, histopathologists interpretating staining results with these markers should take the available clinical data and the histological features of the metastatic carcinoma into consideration. These data are necessary to estimate the relative a priori probability of possible carcinomas. Based on Bayes' theorem, the a priori probability can then be used to calculate the diagnostically relevant predictive values for immunostaining results with the chosen markers.
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PMID:[Immunohistochemical diagnosis in cancer metastasis of unknown primary tumor]. 1208 86

Malignant change in a benign vascular tumor is exceedingly rare, and there have been only five previously reported convincing cases. Four new cases of angiosarcoma (AS) arising in a hemangioma/vascular malformation (HVM) are described. All patients were in the 6th or 7th decade of life (two female, two male). Development of an enlarging deep-seated mass was the main presenting symptom. MRI disclosed the presence of two separate soft tissue masses in both thighs in one patient. No patient had a history of prior radiotherapy at the same site. Preoperative duration, known in three cases, ranged from 1 to 24 months (median 12 months). Three tumors were located in the lower extremities (thigh and buttock), one in the retroperitoneum, and one in the parotid region. Three patients were treated by marginal excision; in one case only a biopsy was performed. Radiotherapy/chemotherapy was given in all cases. Two patients were disease free 2 and 14 months after surgery and two developed metastases. Grossly, the tumors were described as frankly hemorrhagic masses or as firm, whitish areas with hemorrhagic nodules and were centered in skeletal muscle in three cases. Size ranged between 2.2 cm and 8 cm (median 4.3 cm). Histologically, all the tumors had two distinct components. In three cases the benign and the malignant components were variably intermixed, whereas in one case the HVM was mainly located at the edge of the malignant tumor. The benign component showed features of an arteriovenous hemangioma (three cases) or intramuscular capillary hemangioma. AS showed epithelioid morphology in three cases and a well-differentiated dissecting pattern in one case. An imperceptible transition between the two components was noted in two cases. The two anatomically separate masses excised from one patient appeared almost identical. All cases were positive for at least two endothelial markers (CD31, CD34, VWF) and negative for the epithelial markers (EMA, AE1/AE3, Pan-keratin). Possible mechanisms for this exceptional phenomenon are discussed.
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PMID:Angiosarcoma arising in hemangioma/vascular malformation: report of four cases and review of the literature. 1236 47

We report a case of non-functioning adrenal cortical carcinoma (ACC) presenting with metastatic disease to the tongue, which is an extremely uncommon onset for this neoplasm. Histologically, the lesion had the appearance of an anaplastic neoplasm, and a panel of immunohistochemical markers including vimentin, MART-1, S100 protein, HMB-45, smooth muscle actin, common muscle actin, desmin, CD31, CD34, CD68, EMA and cytokeratins, was helpful in excluding melanoma, as well as other mesenchymal and epithelial neoplasms.
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PMID:Non-functioning adrenal cortical carcinoma presenting with metastasis to the tongue. 1258 89

The sclerosing epithelioid fibrosarcoma (SEF), defined as an entity by Meis-Kindblom et al. in 1995 [15] is now considered to be a variant of fibrosing fibrosarcomas. It is a rare tumor with an intermediate malignant potential leading to local recurrences in one third and to metastases in about 40% of the cases. We report six cases of this entity. At the time of diagnosis two patients of our series already showed metastases in the lungs. The tumors were located in the deep soft tissue and measured between 2.5 and 17 cm. The histology is characterized by small epithelioid cells that are arranged individually or in cords and nests and set typically in a hyaline sclerotic matrix. By immunohistochemistry, all cases were vimentin positive, however EMA positive cells are also possible. The differential diagnosis includes metastases of carcinoma, benign and malignant soft tissue tumors. The distinction of SEF from fibromatosis, fibrous histiocytomas, ossifying fibromyxoid tumors, clear cell sarcomas, epithelioid sarcomas, synovial sarcomas and extraskeletal osteosarcomas is discussed.
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PMID:[Sclerosing epithelioid fibrosarcoma]. 1267 98

Placental site trophoblastic tumour (PSTT) is a very rare and unique form of gestational trophoblastic disease (GTD). This tumour represents a neoplastic transformation of intermediate trophoblastic cells that normally play a critical role in implantation. PSTT can occur after a normal pregnancy, abortion, term delivery, ectopic pregnancy or molar pregnancy. It displays a wide clinical spectrum, and when metastatic, can be difficult to control even with surgery and chemotherapy. Unlike other forms of GTD, PSTT is characterized by low beta-hCG levels because it is a neoplastic proliferation of intermediate trophoblastic cells. Expression, however, of human placental lactogen (hPL) is increased on histologic section as well as in the serum. The most common presenting symptoms of PSTT are vaginal bleeding and amenorrhoea. Diagnosis is confirmed by dilatation and curettage (D and E) and hysterectomy but meticulous evaluation of metastasis is mandatory. Most cases are confined to the uterus but pelvic involvement, lung and other organ metastasis has been reported. Unlike other forms of GTD, the WHO prognostic score is of little help. For the PSTT patient, surgery is the primary treatment of choice. For patients desiring future childbearing, D and C and adjuvant chemotherapy is an option. Because these tumours tend to be less sensitive than other types of GTD to chemotherapy, the most successful regimen to date has been with EMA/CO or EMA/EP. Good prognosis is anticipated in cases localized to the uterus, and when the interval between antecedent pregnancy and treatment is less than 2 years. In cases with distant metastasis or delayed treatment, the outcome is dismal. Advances in chemotherapeutic regimens have improved clinical reponse in metastatic disease.
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PMID:Placental site trophoblastic tumour. 1461 93

A 26-year-old woman who had been treated for nonmetastatic gestational trophoblastic tumor with three courses of methotrexate with folinic acid rescue and had been lost to follow up for 4 years was referred with the fractional curettage diagnosis of choriocarcinoma that had been performed for abnormal vaginal bleeding. Her serum beta human chorionic gonadotropin (betahCG) was 706000 mIU/mL and there were multiple pulmonary metastatic foci. The uterus was 12 weeks pregnant-size and a 6 x 6-cm tumor mass was seen within the anterior uterine wall at ultrasonography. Following total abdominal hysterectomy etoposide, methotrexate, actinomycin-D, vincristine and cyclophosphamide (EMA/CO) regimen was given. Whole brain radiation of 30 Gy in 3 weeks for brain metastasis, discovered in magnetic resonance imaging was given after the first course. Since serum betahCG levels plateaued after three courses of chemotherapy and multiple pulmonary metastases persisted, treatment was shifted to etoposide, methotrexate, actinomycin-D, etoposide, cisplatin (EMA/EP) regimen. She was in remission after three courses of chemotherapy.
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PMID:Successful salvage therapy of resistant gestational trophoblastic disease with etoposide, methotrexate, actinomycin-D, etoposide, cisplatin (EMA/EP). 1464 86

We present a series of 5 penile clear cell carcinomas, which arose in middle-aged men at the inner side of the foreskin. They were large, exophytic, partly ulcerated, and widely invasive tumors with sharp demarcation to the surrounding normal skin/mucosa. Histologically, they were composed of large clear cells with intracytoplasmic PAS/d-PAS-positive material and showed extensive lymphatic and blood vessel invasion. Strong staining with antibodies to Muc-1, EMA, and CEA was typical. All carcinomas harbored HPV16 DNA, although only one carcinoma revealed HPV-related cytologic cell changes. All 5 patients had extensive, partly cystic inguinal lymph node metastases with a striking clear cell differentiation and focal dense sclerotic basement membrane material, either at or within several months after initial diagnosis. Two patients are alive without disease after 7 and 10 years. One patient died after 9 months of widespread disease and 2 patients are presently alive at 7 and 17 months follow-up with widespread lymphatic and hematogenous metastases despite adjuvant chemo- and radiation therapy. In contrast to squamous cell carcinoma, penile clear cell carcinomas show extensive blood and lymph vessel invasion and early metastases to regional lymph nodes. Clear cell carcinomas represent a distinct group of penile cancers that may have a different clinical behavior than usual penile squamous cell carcinomas.
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PMID:Penile clear cell carcinoma: a report of 5 cases of a distinct entity. 1548 56

Female adnexal tumor of probable Wolffian origin (FATWO) is a rare entity which is believed to originate from mesonephric (Wolffian) remnants on the basis of its location where the remnants are abundant. Its behavior is usually indolent, although some cases can recur or metastasize. The authors present the clinicopathological features of two cases of FATWO arising in the broad ligament, and focus on the expression of adhesion molecules and proliferative marker. Mesonephric duct remnants are also examined in an attempt to elucidate the histogenesis of FATWOs. The two FATWOs were well-circumscribed solid masses arising in the leaves of the broad ligament and histological examination revealed a mixture of cysts and tubules imparting a sieve-like pattern and mucin-negative eosinophilic secretion within these tubules. Immunohistochemically, the tumors showed the expression of cytokeratin 7 and 20, high-molecular-weight cytokeratin, and calretinin, which closely resembled that of the mesonephric duct remnants. Regarding CK 20, CD 10, EMA, S-100 protein, and vimentin their expression was in part not identical with previous studies. E-cadherin, alpha and beta-catenin were strongly expressed along the cell membrane of the tumor cells. The Ki-67 labeling index of FATWO was 0% and 3.2% in each case. The preservation of the E-cadherin-catenin complex and low Ki-67 labeling index could explain the indolent behavior and low malignant potential of this tumor.
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PMID:Expression of adhesion molecules and Ki-67 in female adnexal tumor of probable Wolffian origin (FATWO): report of two cases and review of the literature. 1551 Dec 77

June 2004: Over the past year, this man in late-60s had complained about progressive weakness of concentration and memory disturbances, associated with word finding difficulties. MRI examination revealed an extra-axial, parasagittal tumor 3 cm in diameter located in the left frontoparietal region. Five years ago, a meningioma in the same region, with radiographic appearance comparable to the present tumor had been totally removed. The histological picture of the current tumor was dominated by sheets of large rounded pleomorphic tumor cells with abundant eosinophilic cytoplasm and eccentric nuclei (rhabdoid cells). Cytoplasmic inclusions were frequent; occasionally,multinucleatedtumorcellswereseen. Mitoticfigures were absent and the MIB was 3%. Meningothelial lobules were scarce, and regions with fibroblastic appearance were absent. There were no psammoma bodies, necrosis or brain invasion. Moderate immunoreactivity for EMA was found. Additionally, strong cytoplasmic immunoreaction for vimentin within the rhabdoid cells was observed. Review of the previous material showed small islets of rhabdoid cells. Rhabdoid meningioma is an uncommon meningioma variant. It has been suggested that rhabdoid meningiomas are highly aggressive tumors (WHO grade III)and that the rhabdoid phenotype represents a marker of malignant transformation in meningiomas. Histologically, rhabdoid meningiomas usually exhibit signs of anaplasia, a high mitotic activity, and a markedly increased MIB-1 labeling index. Extracranial metastases may occur in the course of the disease. However, not all rhabdoid tumors appear to have anaplastic features (as this case illustrates). Another interesting feature of rhabdoid meningiomas is that in a significant number of cases, the rhabdoid cells appear only at the time of recurrence. Alternatively, as seen in this case, the rhabdoid cells may be already present in the primary meningioma, but not as the predominating histological feature.
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PMID:June 2004: a male in his late 60s with recurrent extracerebral tumor. 1560 95


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