Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Endovascular papillary angioendothelioma is a rare vascular neoplasm originally described as involving the skin and subcutaneous tissues. No case has been reported to involve the spleen, although structurally similar splenic neoplasms have been reported under different names. A 5-year-old boy presented with isolated splenomegaly and was found to have endovascular papillary angioendothelioma of the spleen. Studies supported the suggestion that the endovascular papillary angioendothelioma manifests focal "high" endothelial differentiation.
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PMID:Endovascular papillary angioendothelioma in the spleen. 341 2

Papillary angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally. We report the case of a 6-year-old girl with isolated splenomegaly and symptoms of early satiety and weight loss, whom was found to have a splenic papillary angioendothelioma. Preoperative abdominal computed tomography scan showed an irregular, heterogeneous mass; a tagged red cell scan ruled out a hemangioma, whereas a positron emission tomography scan showed mildly increased uptake. Subsequent surgery and pathologic assessment revealed a papillary angioendothelioma (Dabska tumor) within lymphatic spaces. The child has no evidence of recurrence or metastases 1 year postoperatively.
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PMID:Splenic papillary angioendothelioma in a 6-year-old girl. 1809 Sep 26