Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a case of a poorly differentiated monophasic synovial sarcoma arising in the lung of a 50-year-old man. The tumor, which was located in the right upper lobe, was lobulated, relatively well-circumscribed, and whitish to yellowish in color. Microscopically, it was composed exclusively of ovoid to polygonal or short spindle cells, with a high nuclear to cytoplasmic ratio and relatively scant cytoplasm, arranged in solid sheets or in a hemangiopericytomatous pattern with intervening wiry collagen fibers. At the periphery of the tumor, entrapped benign alveolar epithelium produced a pseudo-biphasic appearance. In some areas, an abundance of keloidal collagen imparted a close resemblance to a solitary fibrous tumor, making it difficult to establish the diagnosis on the initial needle biopsy, although the malignant nature of the tumor was suggested because of nuclear anaplasia. Immunohistochemically, the tumor was positive for cytokeratin AE1/AE3, CAM5.2, EMA, vimentin, bcl-2 protein, calretinin, and CD34. The reverse transcriptase-polymerase chain reaction (RT-PCR), using RNA extracted from fresh-frozen tissue, demonstrated SYT/SSX-1 fusion transcripts, confirming the diagnosis of synovial sarcoma. Microscopic examination demonstrated metastatic deposits in hilar lymph nodes. This case indicates that a primary pulmonary synovial sarcoma, particularly in its poorly differentiated form, is a diagnostically challenging and highly aggressive neoplasm typically found at an advanced stage.
 ...
PMID:Primary poorly differentiated monophasic synovial sarcoma of the lung. A case report with immunohistochemical and genetic studies. 1864 80

Angiosarcoma occurs very rarely in the intestinal tract as either a primary or metastatic malignancy and can present great diagnostic difficulty, especially when it displays epithelioid cytomorphology. Since only isolated case reports have been published, the purpose of this study is to more fully delineate the histopathological and clinical features from a series of 8 angiosarcomas involving the gastrointestinal tract. There were 5 male and 3 female patients whose ages ranged from 25-85 years (median 57). Presenting symptoms included intestinal bleeding, anemia and pain. Five cases involved the small bowel and 3 involved the colon/rectum. Four cases were primary to the intestinal tract, 2 patients initially presented with secondary involvement of the large bowel from occult retroperitoneal primaries, 1 patient presented with disseminated disease including small bowel involvement, and 1 case was metastatic from a breast primary. Seven cases were composed predominantly of sheets of malignant appearing epithelioid cells with subtle areas forming cleft-like spaces suggestive of vascular differentiation. Immunohistochemical studies revealed the lesional cells to be immunoreactive for CD31 (8/8), CD34 (8/8), Factor VIII (8/8), cytokeratins AE1/AE3 (7/8), cytokeratin 7 (2/8), Cam5.2/cytokeratin 8 (5/8), and cytokeratin 19 (5/8). Cytokeratin 20 was negative in all eight cases, which contrasts sharply with the characteristic positivity for cytokeratin 20 in virtually all intestinal carcinomas. One case was weakly and focally positive for EMA and all cases were negative for S-100 protein. Cytokeratin staining was variable and ranged from focal to extensive. Follow-up was available in eight cases and ranged from 1-33 months (median 12.5). Five patients died of disease, between 1 and 33 months (median 6) after diagnosis. One recently diagnosed patient is alive with disease 18 months after diagnosis, and one patient is free of disease 27 months after original diagnosis. Angiosarcomas of the gastrointestinal tract commonly display epithelioid cytomorphology, may be diffusely and strongly positive for cytokeratins and only show subtle signs of vascular differentiation, creating potential diagnostic confusion with primary or metastatic carcinoma. Given the clinically aggressive behavior of angiosarcoma, proper classification and treatment is important. Immunohistochemistry with vascular markers, CK20, and S-100 protein may be helpful in differentiating angiosarcoma from carcinoma and melanoma.
 ...
PMID:Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. 1510 92

Based on clinical and histologic features, differentiating metastatic carcinomas from benign or malignant meningiomas usually is not difficult. Occasionally, however, in some patients without a clinical history of carcinoma, malignant meningiomas can morphologically simulate metastatic carcinoma, necessitating an immunohistochemical study for cytokeratin to make a correct diagnosis. However, the utility of immunohistochemical markers to separate malignant meningioma from metastatic carcinoma has not been investigated. The immunoperoxidase method with antigen retrieval was used to characterize the expression of three cytokeratins (AE1/AE3, CAM 5.2, and Pan cytokeratin), EMA, CEA, Ber-EP4, CD 15, and B72.3 in 12 previously diagnosed malignant meningiomas, 20 benign meningiomas, and 20 metastatic carcinomas. Cytokeratin expression was detected in 75% of malignant meningiomas, 0% of benign meningiomas, and 100% of metastatic carcinomas. While epithelial markers of Ber-EP4, CEA, B72.3 and CD-15 were positive in 90, 80, 70 and 65% of the metastatic carcinoma, respectively, they were negative in all 12 malignant meningioma examined. Vimentin immunoreactivity was seen in all benign and malignant meningiomas, and in 20% of metastatic carcinomas. Our results indicated that cytokeratin is not a reliable immunohistochemical marker to separate a malignant meningioma from metastatic carcinoma. A panel of epithelial markers including Ber-EP4, CEA, B72.3 and CD-15, and vimentin may be needed to separate malignant meningioma from metastatic carcinoma. Cytokeratin expression can be a potential pitfall for confusing a malignant meningioma with a metastatic carcinoma.
 ...
PMID:Expression of cytokeratin by malignant meningiomas: diagnostic pitfall of cytokeratin to separate malignant meningiomas from metastatic carcinoma. 1513 78

Ovarian small cell carcinoma of hypercalcemic type (OSCCHT) is a rare neoplasm with an aggressive behavior, broad differential diagnosis, and unknown histogenesis. To add to knowledge concerning the possible aid of immunohistochemistry in resolving problems in differential diagnosis and to further explore whether that modality points to any specific histogenesis, we undertook an immunohistochemical study of this neoplasm. Fifteen OSCCHTs (including four of the ''large cell" variant) were stained with a range of antibodies, some of which have not been investigated previously in this neoplasm. Cases were stained with AE1/3, EMA, BerEP4, CK5/6, calretinin, WT1, chromogranin, CD56, synaptophysin, CD99, NB84, desmin, S100, CD10, alpha inhibin, TTFI, and p53. Staining was classified as 0 (negative), 1+ (<5% cells positive), 2+ (5% to 25% cells positive), 3+ (26% to 50% cells positive), or 4+ (>50% cells positive). All cases were positive with p53 (two 1+, five 3+, eight 4+), 14 of 15 cases were positive with WT1 (one 1+, thirteen 4+), 14 of 15 with CD10 (three 1+, four 2+, two 3+, five 4+), 13 of 15 with EMA (three 1+, three 2+, two 3+, five 4+), 11 of 15 with calretinin (nine 1+, one 3+, one 4+), 9 of 15 with AE1/3 (eight 1+, one 2+), 4 of 15 with CD56 (one 1+, two 2+, one 4+), 3 of 15 with BerEP4 (two 2+, one 4+), 2 of 15 with synaptophysin (two 1+), and 1 of 15 with S100 (4+). All cases were negative with CK5/6, chromogranin, CD99, NB84, desmin, alpha inhibin, and TTF1. The only noticeable difference in the immunophenotype between typical OSCCHT and the large cell variant was that there was 4 +EMA positivity in three of four cases of large cell variant compared with two of 11 cases of typical OSCCHT. OSCCHT is characteristically positive with AE1/3, EMA, CD10, calretinin, WT1, and p53. Combined EMA and WT1 positivity, the latter usually intense and diffuse, may be of diagnostic value, inasmuch as only a few of the neoplasms in the differential diagnosis are positive with both antibodies. Negative staining with CD99, desmin, NB84, alpha-inhibin, and TTF1 may aid in the cases in which primitive neuroectodermal tumor, rhabdomyosarcoma, intraabdominal desmoplastic small round cell tumor, neuroblastoma, a sex cord-stromal tumor, and metastatic pulmonary small cell carcinoma are in the differential. Calretinin positivity precludes its use in the differential with granulosa cell tumors. The results of this investigation do not settle the issue of histogenesis, which remains enigmatic. The typical age distribution, follicle formation, and calretinin positivity are consistent with a sex cord origin. On the other hand, WT1 and EMA positivity and negative staining with alpha-inhibin would be unusual in a sex cord-stromal neoplasm and can be used as an argument for a surface epithelial origin. Germ cell and neuroendocrine origins seem highly unlikely.
 ...
PMID:An immunohistochemical analysis of ovarian small cell carcinoma of hypercalcemic type. 1538 2

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm. The cytological diagnosis of these tumors can be difficult because they show morphological features quite similar to other small round blue cells tumors. We described four cases of DSRCT with cytological sampling: one obtained by fine needle aspiration biopsy (FNAB) and three from serous effusions. The corresponding immunocytochemical panel was also reviewed. METHODS: Papanicolaou stained samples from FNAB and effusions were morphologically described. Immunoreaction with WT1 antibody was performed in all cytological samples. An immunohistochemical panel including the following antibodies was performed in the corresponding biopsies: 34BE12, AE1/AE3, Chromogranin A, CK20, CK7, CK8, Desmin, EMA, NSE, Vimentin and WT1. RESULTS: The smears showed high cellularity with minor size alteration. Nuclei were round to oval, some of them with inconspicuous nucleoli. Tumor cells are clustered, showing rosette-like feature. Tumor cells in effusions and FNA were positive to WT1 in 3 of 4 cytology specimens (2 out 3 effusions and one FNA). Immunohistochemical reactions for vimentin, NSE, AE1/AE3 and WT1 were positive in all cases in tissue sections. CONCLUSION: The use of an adjunct immunocytochemical panel coupled with the cytomorphological characteristics allows the diagnosis of DSRCT in cytological specimens.
 ...
PMID:Desmoplastic small round cell tumour: Cytological and immunocytochemical features. 1577 80

Angiomyofibroblastoma (AMF) is a rare, benign, mesenchymal tumor occurring mainly in the female genital tract. Some cases contain scattered mature adipocytes, but the lipomatous variant in which mature adipose tissue is prominent or striking is rare. Only five cases have been reported in the English literature. We report two more such cases that were composed of 70 to 80% and 30 to 40% adipose tissue, respectively. Immunohistochemical analysis showed that the tumor cells were positive for estrogen receptor, progesterone receptor, vimentin, and Bcl-2, and negative for cytokeratin AE1/1, EMA, and CD117. Tumor cells in the first case were positive for CD34 but not desmin and muscle-specific actin. The opposite expression profile of these three markers was observed in tumor cells in the second case: positive for desmin and muscle-specific actin and negative for CD34. Rare cells were positive for S-100 in adipose-rich areas in the first case. Our findings indicate that the tumor cells in the lipomatous variant have similar immunoprofile to those of usual AMF and support the concept that the lipomatous variant probably represents an extreme end of a wide spectrum of differentiation in AMF.
 ...
PMID:Lipomatous variant of angiomyofibroblastoma: report of two cases and review of the literature. 1578 77

The immunohistochemistry study made on 30 cases of salivary glands pleomorphic adenomas aims to establish the antigen profile of luminal epithelial neoplastic component proliferates into these tumors. We ascertain that luminal epithelial neoplastic cell were almost exclusively positive to antibody peculiar to epithelial differentiation (AE1/AE3, MNF 116, CK 8, CK 19, CK20, EMA) and occasional to S-100 protein and negative to vimentin, alpha-smooth muscle actin, calponin, Glial Fibrillary Acidic Protein (GFAP). This kind of immunoreactivity was similar to ductal cells from striated channels of salivary glands, suggesting the origin of such pleomorphic adenomas from this place.
 ...
PMID:The immunohistochemical profile of luminal epithelial neoplastic component from pleomorphic adenomas of salivary glands. 1584 84

The present immunohistochemical study was carried out on ten cases of ovarian tumours diagnosed with usual staining as undifferentiated ovarian carcinomas. A panel of antibodies was chosen in order to confirm the epithelial origin of these tumours and to eliminate some possible ovarian metastasis. The cytokeratin AE1/AE3, EMA and BerEP4 positivity supported their epithelial origin even if thirty per cent of the cases co-expressed cytokeratin and vimentin. Even if there is not a specific marker to confirm the ovarian origin of the tumours, the CA125 and CEA positivity suggested that these carcinomas might represent a serous or a mucinous dedifferentiation. Associated with these antibodies, the calretinin, CK7 and CK20 stainings allowed the separation of these tumours from peritoneal mesotheliomas with ovarian extension and from ovarian metastasis originating in the gastrointestinal tract. The evaluation of the PCNA labelling index confirms the high degree of cell proliferation and the aggressiveness of these tumours.
 ...
PMID:Value of immunohistochemistry in confirming undifferentiated ovarian carcinomas. 1584 87

An immunohistochemical investigation of 12 primary biphasic synovial sarcomas was carried out. Highly frequent expression of vimentin, EMA and pan-cytokeratin (cytokeratin AE1/AE3) and low frequency of collagen type II and VI expression in the extracellular matrix of tumors were estallished.
 ...
PMID:[Immunohistochemical characterization of biphasic synovial sarcomas]. 1590 13

Sarcomatoid carcinomas are uncommon, high-grade tumors, predominantly composed of spindle cells. Only a few cases arising in the penis have been reported. The aim of this study is to better define the clinicopathologic features of this neoplasm. A total of 400 cases of squamous cell carcinoma of the penis were reviewed from which 15 sarcomatoid carcinomas (4%) were identified. Clinical and pathologic features were evaluated in all cases. Immunohistochemical studies for expression of AE1/AE3, Cam 5.2, 34betaE12, EMA, vimentin, muscle specific actin, smooth muscle actin, desmin, S-100, p63, and p53 and in situ hybridization studies for HPV were performed in 5 cases. Information about lymph node status was available in 9 cases, and follow-up in 5 cases. The mean age was 59 years, and mean tumor size was 5 cm. Grossly, most tumors were large, polypoid, and ulcerated masses frequently affecting the glans (93%) and deeply invading corpora cavernosa (80%) and skin. Microscopically, the lesions were predominantly composed of atypical spindle cells disposed in interlacing fascicles, resembling fibrosarcoma or leiomyosarcoma, sometimes admixed with pleomorphic giant cells mimicking malignant fibrous histiocytoma. One case was predominantly composed of myxoid areas. Less frequent and focal patterns were pseudoangiomatous and epithelioid. Mitotic figures were numerous, and necrosis was prominent. Foci of heterologous differentiation toward bone (osteosarcomatous component) were present in 1 case. Four cases showed a minor mixed component of usual, papillary, verrucous, and basaloid carcinoma. Intrapenile metastasis ("satellitosis") was present in 4 tumors. One of the cases was multicentric with a separate independent focus of well-differentiated carcinoma with pseudohyperplastic features. Associated low- and high-grade squamous intraepithelial lesions were noted in 73% of the cases. Immunohistochemical studies and HPV in situ hybridization were done in 5 cases. The spindle cells were diffusely positive for vimentin and p53 and showed at least intermediate expression of 34betaE12 and p63 in all cases. EMA and AE1/AE3 were focally positive in 60% of the cases, and Cam 5.2 was focally positive in 1 case. Tumor cells failed to express muscle specific actin, smooth muscle actin, desmin, and S-100. HPV in situ hybridization was negative in all cases. Inguinal metastases were present in 89% of the cases. Two of five patients with adequate follow-up died of disease within 8 months of the diagnoses. In conclusion, penile sarcomatoid carcinomas are unusual, large, and aggressive tumors usually associated with lymph node metastasis and poor outcome. Differential diagnoses include sarcoma and melanoma. Cytokeratin 34betaE12 and p63 appear to be the more specific and sensitive markers to categorize these tumors as epithelial. Diffuse immunoreactivity for p53, compared with a more basal and focal reactivity in differentiated squamous cell carcinoma, may be indicative of a late mutation in the natural progression of the disease.
 ...
PMID:Sarcomatoid carcinoma of the penis: a clinicopathologic study of 15 cases. 1609 3


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>