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一名儿科患者的胃肠道肠套叠和平滑肌肉瘤。病例报告及文献复习。

Intussusception and leiomyosarcoma of the gastrointestinal tract in a pediatric patient. Case report and review of the literature.

作者信息

Furuta G T, Bross D A, Doody D, Kleinman R E

机构信息

Combined Program in Pediatric Gastroenterology and Nutrition, Massachusetts General Hospital, Boston 02114.

出版信息

Dig Dis Sci. 1993 Oct;38(10):1933-7. doi: 10.1007/BF01296122.

Abstract

Intestinal leiomyosarcoma is a rare tumor in infants and children; only 46 cases have been reported in the English literature. Presenting signs and symptoms include abdominal pain and gastrointestinal obstruction and bleeding. We describe a neonate with the unique presentation of ileocecal intussusception accompanying an ileal leiomyosarcoma. In contrast to adult patients, where intussusception is associated with smooth muscle tumors in 30% of cases, leiomyosarcoma and subsequent intussusception is rare in infancy and childhood. The overall prognosis for long-term survival is similar for both pediatric and adult patients with leiomyosarcoma of the intestinal tract, with a five-year survival in reported cases of 53% and 40%, respectively.

摘要

肠道平滑肌肉瘤在婴幼儿中是一种罕见肿瘤;英文文献中仅报道过46例。其呈现的体征和症状包括腹痛、胃肠道梗阻及出血。我们描述了一名患有回盲部肠套叠并伴有回肠平滑肌肉瘤这一独特表现的新生儿。与成年患者不同,成年患者中30%的肠套叠与平滑肌瘤有关,而平滑肌肉瘤及随后发生的肠套叠在婴幼儿及儿童期较为罕见。患有肠道平滑肌肉瘤的儿童和成年患者的长期总体生存预后相似,报道病例中的五年生存率分别为53%和40%。

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