Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Differentiation of reactive and/or atypical mesothelial cells from malignant epithelial cells in serous effusions remains a frequent diagnostic problem. Since epithelial membrane antigen (EMA) positive malignant cells in serous effusions have been reported in almost all adenocarcinomas and most malignant mesotheliomas, immunoreactivity for EMA is felt to be less useful than other antibodies in the workup of problematic serous effusions. However, immunostaining of reactive and/or atypical benign mesothelial cells for EMA has not been well studied, with only a few series reporting either weak or negative staining for EMA. This study was undertaken to evaluate how often reactive and/or atypical appearing mesothelial cells stain positively for EMA. One hundred eighty serous effusions (115 pleural, 55 peritoneal, and 10 pericardial) from 123 females and 57 males ages 20 to 89 yr were evaluated in which an antibody panel including EMA was performed on cell blocks (141 cases), cytospins (36 cases), or both (3 cases). Of the 100 cytologically positive cases, EMA immunoreactivity was present in 97/100 (97%) cases. One EMA negative case suspicious for a metastatic renal cell carcinoma was lost to follow-up and not included in the analysis. The remaining three negative cases consisted of malignancies not expected to have EMA positive cells (small cell carcinoma, neuroblastoma, and synovial sarcoma). Therefore, EMA was positive in virtually 100% of the remaining malignant cases. In the 78 cytologically negative cases, EMA positivity was present in 3/78 (3.8%) cases. Clinical follow-up of up to 14 mo in these three cases revealed no evidence of malignancy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Significance of epithelial membrane antigen in the work-up of problematic serous effusions. 758 72

We report the case of a 5-year-old girl with metastatic renal cell carcinoma (RCC) diagnosed 19 months after treatment for neuroblastoma. Immunostaining of the secondary tumor was consistent with Xp11 translocation morphology. This is the second report of this translocation RCC presenting after neuroblastoma and the most rapid onset of RCC reported thus far. The literature regarding secondary RCC after neuroblastoma is reviewed and our case is placed within this historical context. As our understanding of the genetic changes in pediatric tumors advances, the reporting of these rare cases with specific emphasis on genetic testing provides a resource for clinicians and researchers.
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PMID:Rapid development of metastatic Xp11 translocation renal cell carcinoma in a girl treated for neuroblastoma. 1963 62

Primary primitive neuroectodermal tumors (PNETs) of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm's tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES)/PNET have a specific t(11;22) which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient's condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.
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PMID:Primary primitive neuroectodermal tumor of kidney: a rare case report with diagnostic challenge. 2494 71