Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of a neonate presenting with the rare vascular tumor, Kaposiform hemangioendothelioma. She had a lesion arising from the left ovary with multiple intraperitoneal metastases causing small bowel obstruction. We managed this case with primary surgical resection followed by laparoscopic surveillance of the peritoneal cavity and metastectomy. The child is fit and well and free of tumor after 32 months. Neither this presentation of this tumor nor this management approach has been previously described in the literature.
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PMID:Intra-abdominal Kaposiform hemangioendothelioma and the benefits of laparoscopic surveillance. 2243 Dec 47

Kaposiform haemangioendothelioma is a rare, locally aggressive vascular tumour, which is mainly found in children. Typically, it occurs in the skin. The tumour is borderline malignant due to its rapid infiltrative growth. Metastasis has not been described. Since 1993, when this tumour was first defined, less than 160 cases have been reported. To our knowledge, this is the first case of kaposiform haemangioendothelioma in the gastrointestinal tract. We report a case of a three year-old boy, who presented with small bowel obstruction due to invagination of a kaposiform haemangioendothelioma.
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PMID:[Kaposiform haemangioendothelioma in the small intestine of a three year-old boy]. 2268 97

Kaposiform hemangioendothelioma (KHE) is a rare and locally aggressive vascular tumor with histological features resembling Kaposi sarcoma and capillary hemangioma mainly occurring in children and adolescents. Approximately 200 cases have been reported since its original description in 1993, with the vast majority presenting at an early age as raised ill-defined lesions with a red-blue hue mainly involving the skin and soft tissues in the extremities. Cases in adults remain extremely rare. Herein, we describe the case of a 29 year-old man who presented with progressive abdominal pain for 4 months and signs of obstipation found to be consistent with small bowel volvulus. The patient underwent exploratory laparotomy and resection of 55 cm of necrotic small bowel followed by enteroenterostomy and anastomosis. Microscopic examination revealed KHE involving small intestinal mesentery, muscularis propria, and submucosa. His recovery was uneventful and he was discharged after stabilization, opting to manage him expectantly with abdominopelvic imaging and to monitor for development of Kasabach-Merritt phenomenon. To our knowledge, this represents the first reported case of this entity presenting as intestinal obstruction in an adult for which we also present a review of the existing literature and possible treatment options.
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PMID:Kaposiform Hemangioendothelioma of the GI Tract: An Exception to Occam's Principle in an Adult with SBO. 3109 93