Synchronous gastrointestinal stromal tumors (GIST) and other primary cancers: Case series of a single institution experience
Synchronous gastrointestinal stromal tumors (GIST) and other primary cancers: Case series of a single institution experience
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DOI：
10.1016/j.ijsu.2010.03.008
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发表时间：
2010-01-01
影响因子：
15.3
通讯作者：
Garicochea, Bernardo
中科院分区：
文献类型：
--
作者：
Ferreira, Sheila S.;Werutsky, Gustavo;Garicochea, Bernardo
背景：胃肠道间质瘤（GIST）是一种罕见的累及胃肠道的间叶性肿瘤。间叶肿瘤和其他原发性肿瘤的偶然发生还没有很好地描述在literature.Objective：本研究的目的是评估GIST的临床和病理特征，同时发生与其他原发性tumors.Methods：43例诊断为GIST手术治疗的根治性意图在我们的机构从1998年至2006年。结果：43例患者中，有6例（14%）同时发现GIST和其他原发肿瘤。胃肠道间质瘤位于胃（50%）和小肠（50%），大小为0.7 ~ 7.6cm（中位数3.35cm）。5例（83%）并发原发性肿瘤来自胃肠道，仅1例（17%）患者并发乳腺癌和GIST。GISTs免疫表型中CD 117和CD 34阳性率为100%，平滑肌肌动蛋白（SMA）阳性率为67%，S100阳性率为50%，结蛋白阳性率为33%。而细胞角蛋白AE 1/AE 3和PDGF均为阴性。根据GIST的侵袭行为的风险分类三个被归类为非常低，一个中间和两个high.Conclusions：GIST和其他原发性肿瘤的同时发生是不是一个罕见的实体，通常是偶然发现。胃肠道上皮性肿瘤是最相关的伴随GIST。GIST与同步性肿瘤发生发展的分子和遗传机制有待进一步研究。(C)2010 Surgical Associates Ltd.由Elsevier Ltd.出版。保留所有权利。
Background: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasm affecting the gastrointestinal tract. The incidental occurrence of mesenchymal tumors and other primary tumors has not been well described in literature.Objective: The aim of this study was to evaluate the clinical and pathologic features of GIST occurring synchronously with other primary tumors.Methods: Forty-three patients with diagnosis of GIST treated surgically with curative intent at our institution from 1998 to 2006 were included. The patient clinical data and pathological reports were reviewed.Results: Of the 43 patients, there were 6 (14%) cases of synchronous GIST and other primary tumors discovered as coincidental findings. The synchronous GISTs analyzed were located in the stomach (50%) and small intestine (50%), size ranging from 0.7 to 7.6 cm (median 3.35 cm). Five (83%) of the concurrent primary tumors were from gastrointestinal origin and only one (17%) patient presented with concurrent breast cancer and GIST. The synchronous GISTs immunofenotype shows positivity for CD117 and CD34 (100%), smooth-muscle actin (SMA) (67%), S100 (50%) and desmin (33%). Whereas staining for cytokeratin AE1/AE3 and PDGF were all negative. According to GIST risk category for aggressive behavior three were classified as very low, one intermediate and two high.Conclusions: The synchronous occurrence of GISTs and other primary neoplasm is not an uncommon entity and usually they are discovery incidentally. Epithelial tumors of the gastrointestinal tract are the most associated with concomitant GISTs. Further studies are required to clarify the molecular and genetic mechanisms of carcinogenesis and progression associating GIST and synchronous tumors. (C) 2010 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.