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一例纵隔恶性纤维组织细胞瘤尸检病例,伴有小肠和脑的多发转移——罕见病例报告并文献复习

An autopsy case of malignant fibrous histiocytoma of the mediastinum, presenting multiple metastases to the small intestine and to the brain--a rare case report with a review of the literature.

作者信息

Misonou J, Kanda M, Shishido T, Abe M, Miyake T, Kawamura T, Maekawa I, Itou N, Atsuta T, Kubota H

机构信息

Department of Pathology, Asahikawa City General Hospital, Japan.

出版信息

Gastroenterol Jpn. 1990 Dec;25(6):746-52. doi: 10.1007/BF02779190.

Abstract

An autopsy case of malignant fibrous histiocytoma (MFH) of the mediastinum in a 25-year-old Japanese man is described. He initially complained of general fatigue and intermittent tarry stool, and was radiographically revealed to have a huge mass within the mediastinum as well as multiple nodules within the abdominal cavity, respectively. The mediastinal tumor could not be resected because of direct tumor invasion into surrounding tissues. Within the abdominal cavity, three distinct tumor nodules were discovered in the jejunum, which could be resected surgically. Histopathologically, the components of both lesions were very similar, and the present case was interpreted as multiple metastases of mediastinal MFH to the intestinal wall. In spite of various kinds of treatment, the mediastinal tumor rapidly enlarged, causing SVC syndrome. Brain CT findings suggested multiple metastases which were considered to be the cause of death. Autopsy confirmed that the main tumor nodule was located within the mediastinum, with brain metastases. The present case of mediastinal MFH is considered to be the youngest case as well as the first case presenting intestinal metastases.

摘要

本文描述了一名25岁日本男性纵隔恶性纤维组织细胞瘤(MFH)的尸检病例。他最初主诉全身乏力和间歇性柏油样便,影像学检查分别显示纵隔内有巨大肿块以及腹腔内有多个结节。由于肿瘤直接侵犯周围组织,纵隔肿瘤无法切除。在腹腔内,空肠发现了三个不同的肿瘤结节,可通过手术切除。组织病理学检查显示,两个病变的成分非常相似,本病例被解释为纵隔MFH向肠壁的多发转移。尽管进行了各种治疗,纵隔肿瘤仍迅速增大,导致上腔静脉综合征。脑部CT检查结果提示多发转移,被认为是死亡原因。尸检证实主要肿瘤结节位于纵隔内,并伴有脑转移。本纵隔MFH病例被认为是最年轻的病例,也是首例出现肠道转移的病例。

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