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)

We report a case of a 60-year-old female with a pigmented microcystic chromophobe renal cell carcinoma (PMCRCC). The tumor was 4.5 cm in diameter, and was located in the right kidney. Grossly, on cross section, the tumor was light gray with multiple small brown to black pigmented foci up to 0.2 cm in diameter. Histologically, the tumor showed a microcystic arrangement with cribriform areas and formation of adenomatous structures. The microcystic and cribriform areas were composed of larger pale cells and smaller eosinophilic cells, with cytological features of conventional chromophobe renal cell carcinoma (CRCC). The cytological features of the cells within the adenomatous structures were different. These cells were mostly columnar with nuclei at the base, and had a variable amount of pale to eosinophilic cytoplasm. There were foci of ample brown pigmentation located in the cytoplasm of the tumor cells and extracellularly. In addition, microscopic calcifications were present. Immunohistochemically, the tumor cells were positive for EMA, E-cadherin, cytokeratin CAM5.2, and cytokeratin AE1/AE3. Cytokeratin 7 was positive only focally. S-100 protein, melan A, HMB 45, vimentin, and CD117 were negative. PMCRCC is a rare tumor. To the best of our knowledge, only one series containing 20 cases of this variant of CRCC has been described to date. The important feature is that PMCRCC seems to have a relatively benign biological behavior, and distant metastases and sarcomatoid transformation are absent.
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PMID:Pigmented microcystic chromophobe renal cell carcinoma. 1765

Primary myoepithelial tumors of soft tissue are uncommon, and criteria for malignancy among these neoplasms have only recently been established. Of 51 myoepithelial carcinomas of soft tissue in the literature, 11 occurred in children, 7 of which were included in a previous series of myoepithelial tumors from our group. We have collected an additional 22 cases of myoepithelial carcinoma of soft tissue in the pediatric population, and we describe the detailed clinicopathologic features of all 29 cases herein. There were 15 girls and 14 boys; age at diagnosis ranged from newborn to 17 years (median, 9 y). Sites included extremities (14 cases), trunk (6 cases), viscera (5 cases: 3 mediastinal, 1 retroperitoneal, and 1 intracardiac), and head/neck (4 cases). Histologically, the tumors were heterogeneous, with epithelioid, clear, spindle and/or plasmacytoid cells forming nests, cords or solid sheets in a myxoid or hyalinized stroma. Epithelioid cells predominated in the majority of cases (27 of 29; 93%) and in 10 cases (34%), tumor cells focally had scant cytoplasm with round cell morphology. The mitotic rate ranged from <1 to 68 per 10 high power fields (median, 8), and tumor necrosis was present in 14 cases. At least 1 broad-spectrum cytokeratin was positive in all tumors [CAM5.2 in 17 of 18 (94%), AE1/AE3 in 15 of 20 (75%), and PAN-K in 14 of 21 (67%)], and EMA was positive in 19 of 29 cases (66%). Either S100 or GFAP was positive in all but 4 cases [S100 in 21 of 29 (72%) and GFAP in 15 of 28 (54%)]. Clinical follow-up in 23 cases revealed that 9 patients had local recurrences (53% of the 17 patients who underwent complete excision with negative margins); 12 (52%) developed metastases; and 10 (43%) have died of disease so far, at a median interval of 9 months after diagnosis. Despite the relative rarity of carcinomas in the pediatric population, myoepithelial carcinoma seems to be disproportionately common among children and often has an aggressive clinical course.
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PMID:Myoepithelial carcinoma of soft tissue in children: an aggressive neoplasm analyzed in a series of 29 cases. 1804 35

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

The clinical introduction of new methods for processing fluid samples and the application of supplementary methods for improving the diagnostic accuracy of the pattern of pleurisy is very important for differential diagnosis. The possibilities of using immunocytochemical assay in the practical work of a clinical diagnostic (cytological) laboratory were studied in 96 patients, including 78 and 18 patients with pleural and ascitic fluids, respectively). A Cytospin-IV centrifuge was used for immunocytochemical assay by the routine procedure. The Streptadivin-biotin LSAB2 and EnVision+ test systems were employed to visualize an antigen/antibody reaction. Diaminobenzidine (DAB) was used as a chromogen. A set of markers, comprising 11 antibodies, was applied to the verification of a neoplasm from serous cavities. Mesothelioma was diagnosed in 65 patients. Epithelial mesothelioma was identified in 62 (95.4%) cases. Mesothelioma cells were positive to vimentin and ceratins, calretinin, mesothelin, and thrombomodulin. In 31 cases, adenogenic carcinoma metastases to the serous cavities were typified by an immunopositive reaction to CEA, Ber-EP4, EMA, and cytokeratins and a negative reaction to calretinin, mesothelin, and thrombomodulin. There was occasionally a positive reaction to CD-15 and vimentin.
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PMID:[Use of immunocytochemical assays in the study of exudates from serous cavities in the practical work of a laboratory]. 1831 75

A 77-year-old man visited our department with a painless mass in the left scrotum. He underwent left high orchiectomy with clinical diagnosis of testicular tumor. The tumor penetrated deeply into the internal inguinal ring. The tumor was resected as proximally as possible with positive surgical margin at the internal ring. Macroscopically, the left testis was intact in the resected specimen. Microscopic examination revealed that the tumor was consisted of myxoid stroma with spindle cells, and there was a large variation in histological findings. Immunohistchemical staining was positive with vimentin, and negative with alphaSMA, S-100, Myo D1, EMA, keratin, HMB45, and LCA. Accordingly, the tumor was diagnosed as myxofibrosarcoma, high-grade, of the spermatic cord. Postoperative CT scanning revealed multiple retroperitoneal lymph node metastases. He underwent radiation therapy, but soon developed lung metastases and pleural dissemination, and died 2 months after the operation. Myxofibrosarcoma, which had been called myxoid MFH is one of the most common soft tissue sarcomas. Myxofibrosarcoma or myxoid MFH of the spermatic cord is a rare disease. To our knowledge, only 7 cases have been reported in Japan to date.
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PMID:[Myxofibrosarcoma of the spermatic cord: a case report]. 1840 85

Adamantinomas are rare, low-grade malignant intra-osseous tumors composed of epithelial and mesenchymal elements, which show a marked predilection for the tibia and fibula of young adult male patients. Although cases of adamantinoma located to the axial skeleton have been reported either as recurrent or metastatic disease, only two cases of primary adamantinoma located to the thoracic wall have been previously described. In this study we present the clinical, radiological and histopathological features of a 24-year-old male with a slow growing, solid-cystic, painful mass, located to the right 11th rib, which was morphological and immunohistochemically diagnosed as a primary classic adamantinoma. Radiological studies showed a multiloculated lesion with a solid component. The patient underwent a whole surgical resection of the lesion. Histologically, multiple foci of epithelial cells with basaloid and squamous components were found intermixed within a fibrous stromal tissue. Immunohistochemical analysis demonstrated expression of cytokeratins, EMA, vimentin and other epithelial markers. Primary affection of the rib is an unusual feature of classic adamantinomas.
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PMID:Primary adamantinoma of the rib. Unusual presentation for a bone neoplasm of uncertain origin. 1840 22

Placental site trophoblastic tumor is a rare form of gestational trophoblastic disease, derived from invasive implantation site (intermediate) trophoblastic cells. It is frequently resistant to chemotherapy. Patients with metastases, however, frequently have progressive disease and die despite surgery and multiagent chemotherapy. In this case, a 24-year-old woman was referred because of intermittent vaginal bleeding episodes for 5 months following delivery. Multiple metastases in lungs, liver, kidneys, breast, pancreas, and adrenal and thyroid glands were detected. Combination therapy including surgery and multiagent chemotherapy was planned. Hysterectomy and pelvic lymph node dissection were performed. All metastatic lesions disappeared with EMA-CO treatment. However four courses of BEP regimen, salvage therapy, was performed for plateauing hCG level. Surgery and multiagent chemotherapy seem mainstay of treatment of cases having multiple metastases of PSTTs.
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PMID:Placental site trophoblastic tumor with multiple metastases and complete response to salvage BEP regimen: a case report and review of the literature. 1850 43

Malignant rhabdoid tumor, first described in the kidney of young infants, is a rare and highly aggressive neoplasm of controversial histogenesis that has been reported at many other sites, including the gastrointestinal tract. However, malignant rhabdoid tumor of the small intestine is very rare, with only seven cases published to date. We report a 70-year-old man who presented with abdominal pain and weight loss, and showed a perforated jejunal mass with disseminated metastases by imaging. The patient underwent partial jejunectomy and biopsy of a liver metastasis. Microscopically, the tumor was characterized by neoplastic cells with vesicular nuclei, large nucleoli and abundant eccentric cytoplasm with hyaline globular intracytoplasmic inclusions. Immunohistochemically, the neoplasm coexpressed vimentin and epithelial antigens (AE1/AE3, Cam 5.2, CK34betaE12, CK19 and EMA), most of them showing a peculiar immunostaining pattern in relation to the globular inclusions. Ultrastructurally, the inclusions corresponded to paranuclear whorls of intermediate filaments. The patient received postoperative chemotherapy but died 9 months after surgery. In summary, we report the exceptional case of an undifferentiated carcinoma of the jejunum with rhabdoid phenotype. As with tumors at other sites, recognition of rhabdoid morphology in small intestine neoplasms is of significance because the prognosis is extremely poor.
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PMID:Undifferentiated carcinoma of the jejunum with extensive rhabdoid features. Case report and review of the literature. 1913 90

Metastases from malignancies of the female genital tract to the tonsils have never been reported. A case of a 55-year-old woman presenting with a palatinate tonsil tumour two and half years after primary diagnosis of endometrioid endometrial adenocarcinoma (FIGO Stage IB, G2) and six months after local disease recurrence is presented. The tonsillar malignancy was poorly differentiated and tumour cells were immunohistochemically positive to LMW keratin and EMA, and negative to HMW keratin and LCA, strongly suggesting a possible endometrial origin of the tumour. Metastatic disease was treated with systemic chemotherapy, but the patient soon succumbed due to rapid disease progression. In conclusion, a unique case of a palatinate tonsil tumour as the first metastatic site in an endometrial cancer patient is reported.
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PMID:A unique case of palatinate tonsil metastasis from endometrial cancer. 1948 Feb 65

Clinical investigations with imaging- and endoscopic techniques in order to identify the primary tumor sites in patients with CUP syndrome generally entail a significant diagnostic effort. If costs exceed <euro> 800.00, a financial loss ensues for German hospitals, as public health insurance companies do not reimburse above this amount. Combined cytological/immunocytochemical investigation of metastatic cancer cells represents a cost-effective, minimally invasive procedure to identify the probable primary cancer site that can be applied on an outpatient basis. We report on 85 fine needle aspiration biopsies of metastases to the liver, 30 to the lymph nodes and over 180 serous effusions and/or ascites with metastatic cancer cells in CUP. After conventional microscopic inspection, a routine panel of six monoclonal antibodies was applied (CK 5/6, CK 7, CK 20, Cdx 2, TTF 1 and CA 125). We were thus able to correctly identify the primary tumor sites in 90.3%, 92.0% and 85.1%, respectively, within three days. In total, 23 primary hepatocellular carcinomas could all be classified correctly, applying the antibodies HepPar 1, BerEp 4, AFP, CD 31, CD 68 and Ki 67. In addition, 141 malignant epithelial mesotheliomas were typed correctly in 97.1%, using the antibodies BerEp 4, Calretinin, Mesothelin, EMA and WT. Therefore, immunocytochemical investigation of metastatic cancer cells from fine needle aspiration biopsies or in serous effusions offers an efficient, cost-effective diagnostic alternative to imaging and endoscopic techniques in the workup of patients with CUP syndrome.
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PMID:[Immunocytochemical identification of carcinomas of unknown primaries on fine-needle-aspiration-biopsies]. 1975 20


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