Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0007095 (carcinoid)
 6,990 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although primitive neuroectodermal tumor (PNET) is a well-recognized entity, its renal localization as a primary site has not been appreciated. Only nine cases of renal PNET exist in the literature. The paucity of renal PNET could be explained by the lack of objective diagnostic techniques that would facilitate its distinction from other primitive round cell tumors of the kidney, such as the more widely recognized monophasic Wilms' tumor and clear-cell sarcoma of the kidney (CCSK), as well as renal carcinoid, or neuroblastoma invading the kidney from the adjacent adrenal gland. The recently identified specific fusion transcripts detectable by reverse transcription polymerase chain reaction (RT-PCR) have provided us with a valuable tool for the detection of renal PNET. This article reports three renal PNET that expressed EWS/FLI-1 fusion transcripts by RT-PCR, in addition to positive staining for MIC2 protein and neuron-specific enolase (NSE). One also exhibited dense core granules in cell processes by electron microscopy. Employment of such methodology will lead to a more accurate classification of renal tumors.
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PMID:EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney. 922 42

We report a case of type-B3 thymoma manifesting neuroendocrine differentiation. The patient was a 42-year-old woman who complained of shoulder pain but had no symptoms of myasthenia gravis or anemia. The tumor was located in the anterior mediastinum and had directly invaded the pericardium and left lung. Histological examination revealed that the tumor was lobulated by bands of fibrous tissue, perivascular spaces were scattered throughout the tumor, and there were a few intraepithelial lymphocytes. The vast majority of lymphocytes in the perivascular spaces and in the lobulated tumor were immunohistochemically positive for TdT, MIC2, and CD1a. The majority of tumor cells were polygonal and medium or large in size. The tumor cells were weakly positive for synaptophysin, chromogranin A, CD56, and NSE. Small nests of small, relatively uniform polygonal cells were observed facing the fibrous bands. These cells resembled the cells of carcinoid tumors and were strongly positive for NSE, synaptophysin, chromogranin A, and CD56. Ultrastructurally, sparse dense-core granules were observed in the cytoplasm of a few tumor cells. This is a unique case of thymoma with neuroendocrine differentiation, and to the best of our knowledge this is the first such case ever reported.
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PMID:Atypical thymoma (WHO B3) with neuroendocrine differentiation: report of a case. 1667 17

Mediastinal masses present challenging problems in thoracic practice. Most of them remain asymptomatic for long and by the time the pressure symptoms develop, these are quite advanced. Carcinoids arising from the mediastinum are invariably related to thymus. Non-thymic origin of mediastinal carcinoids is rare, especially in the posterior mediastinum. Only two cases of posterior mediastinal carcinoids have been reported so far. These were assumed to be arising from ectopic thymus tissue. We report a case of a 45-year-old woman who presented with dyspnoea and dry cough due to giant carcinoid tumour of the mediastinum, the pedicle originating from the posterior mediastinum, not related to thymus. She underwent thoracotomy and resection that provided relief. The immunochemical studies revealed positive reaction to cytokeratin, chromogranins and synaptophysin, and negative reaction to S100, CD99 (MIC2) confirming the tumour being neuroendocrine in nature.
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PMID:Primary carcinoid of posterior mediastinum: truth or myth! 2130 2