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[空肠恶性神经鞘瘤伴多发神经纤维瘤及甲状旁腺原发性腺瘤]

[A malignant schwannoma of the jejunum associated with multiple neurofibromas and a primary adenoma of the parathyroid].

作者信息

Godlewski G, Sawan S, Targhetta P, Pignodel C, Marty-Double C, Gaujoux A F

机构信息

Service de Chirurgie Digestive, CHR Carémeau, Nîmes.

出版信息

Ann Gastroenterol Hepatol (Paris). 1989 Jan-Feb;25(1):13-7.

PMID:2496638
Abstract

A case of schwannosarcoma associated with a pleural neurofibroma and a parathyroid adenoma is presented. The neuroectodermal origin and the relationship with neurofibromatosis are discussed. The sarcomatous relapse of a schwannoma previously classified as benign confirms the difficulty of microscopic analysis and the malignancy power of the tumor. A long survival is obtained by a large intestinal and mesenteric resection as long as hepatic metastases are not present or can be resected. The pleural neurofibroma needs symptomatic treatment while parathyroid adenoma must be early removed as soon as malignant hypercalcemia is detected.

摘要

本文报告1例伴有胸膜神经纤维瘤和甲状旁腺腺瘤的神经鞘肉瘤。讨论了其神经外胚层起源以及与神经纤维瘤病的关系。先前被分类为良性的神经鞘瘤发生肉瘤样复发,证实了显微镜分析的困难以及肿瘤的恶性潜能。只要不存在肝转移或可切除肝转移,通过大肠和肠系膜切除术可获得较长生存期。胸膜神经纤维瘤需要对症治疗,而一旦检测到恶性高钙血症,甲状旁腺腺瘤必须尽早切除。

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