Liau C T, Yang T S, Wang C H, Hsueh S
Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, ROC.
Anticancer Drugs. 1996 Feb;7(2):228-32.
Two young adults that presented with intra-abdominal desmoplastic small cell tumors (DSCT) without any evidence of a primary site are described. Both cases share the clinical characteristic features of this rare tumor which include predominant intra-abdominal location as initial presentation, nesting pattern of growth, intense desmoplastic reaction, immunohistochemical reactivity for epithelial, neural and muscle markers, and highly aggressive behavior. Aggressive chemotherapy with a cisplatin-containing regimen was the main therapy to our patients. Up to the present, both cases are alive with disease. The survival is 18 and 15 months from the initial diagnosis, respectively. Interestingly, one of the cases encountered an episode of cerebral infarction at the territory of the left middle cerebral artery 12 days after the first cycle of chemotherapy. This is a previously unrecognized manifestation for this tumor type. This causal relationship between chemotherapy and an acute vascular event is the most likely explanation for our patient's stroke.
本文描述了两名年轻成人患者，他们患有腹内促结缔组织增生性小细胞瘤（DSCT），但未发现任何原发部位的证据。这两个病例都具有这种罕见肿瘤的临床特征，包括以腹内为主的初始表现、巢状生长模式、强烈的促结缔组织增生反应、上皮、神经和肌肉标志物的免疫组化反应性以及高度侵袭性的行为。含顺铂方案的积极化疗是我们患者的主要治疗方法。截至目前，两个病例均带瘤存活。自初次诊断以来的生存期分别为18个月和15个月。有趣的是，其中一名患者在化疗第一周期后12天，左侧大脑中动脉供血区发生了一次脑梗死。这是这种肿瘤类型以前未被认识到的表现。化疗与急性血管事件之间的这种因果关系最有可能解释我们患者的中风。
