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1例复发性腹腔内小儿硬纤维瘤接受6次手术干预

[A case of recurrent intra-abdominal pediatric desmoid tumor undergoing surgical intervention for 6 times].

作者信息

Kametaka Hisashi, Makino Hironobu, Koyama Takashi, Seike Kazuhiro, Hasegawa Akio

机构信息

Dept. of Hepato-Gastrointestinal Surgery, Odawara Municipal Hospital.

出版信息

Gan To Kagaku Ryoho. 2011 Nov;38(12):2523-5.

Abstract

A 15-year-old male patient with palpable abdominal tumor presented to our hospital. CT scan revealed a giant tumor, 15 cm in diameter, with infiltration to the pancreas body. In addition, the tumor invaded to the greater curvature of the stomach and the transverse colon. We performed distal gastrectomy, distal pancreatectomy, splenectomy and transverse colectomy. The final diagnosis of histopathology was desmoid tumor. The tumor recurred locally 9 months after the surgery. Recurrent legion was unresectable because of the invasion to the orifice of SMV and weekly combination chemotherapy of VLB and MTX was started. Although a partial response was achieved for a local recurrent legion after 20 courses, CT scan showed other new recurrent multiple lesions in the abdominal cavity. Due to the severe abdominal discomfort and intestinal obstruction, a reduction surgery was performed 5 times in total. Endocrine therapy and administration of NSAID were not effective. Tumor progression was uncontrollable, and the patient died 5 years and 8 months after the initial surgery.

摘要

一名15岁男性患者因可触及腹部肿物就诊于我院。CT扫描显示一个直径15厘米的巨大肿瘤,侵犯胰体。此外,肿瘤侵犯胃大弯和横结肠。我们实施了远端胃切除术、远端胰腺切除术、脾切除术和横结肠切除术。组织病理学最终诊断为硬纤维瘤。术后9个月肿瘤局部复发。由于侵犯到肠系膜上静脉开口,复发灶无法切除,遂开始每周使用长春碱(VLB)和甲氨蝶呤(MTX)联合化疗。尽管20个疗程后局部复发灶取得部分缓解,但CT扫描显示腹腔出现其他新的多发复发灶。由于严重的腹部不适和肠梗阻,总共进行了5次减瘤手术。内分泌治疗和非甾体抗炎药治疗均无效。肿瘤进展无法控制,患者在初次手术后5年8个月死亡。

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