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一名转移性胃肠道间质瘤(GIST)患者出现副肿瘤表现的不寻常组合。

Unusual combination of paraneoplastic manifestations in a patient with metastatic gastrointestinal stromal tumor (GIST).

作者信息

Tsikrikas Spyridon, Manolakopoulos Spilios, Deutsch Melanie, Alexakis Georgios, Sialevris Konstantinos, Giannopoulos Dimitrios, Vassilopoulos Dimitrios, Archimandritis Athanasios J

机构信息

2nd Academic Department of Internal Medicine, University of Athens, Hippokration General Hospital, Athens, Greece.

出版信息

Scand J Gastroenterol. 2008 Aug;43(8):1012-5. doi: 10.1080/00365520801886090.

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Activating mutations in tyrosine kinase receptors KIT or platelet-derived growth factor receptor alpha (PDGFRA) are the main mechanisms causing the disease. Patients generally present with non-specific symptoms, while a number of tumors are discovered incidentally and may be metastatic at the time of diagnosis. Aggressive GISTs have a defined pattern of metastasis to the liver or throughout the abdomen, or both. Though GISTs rarely present systemic or isolated paraneoplastic reactions, a few cases have been reported in the literature. We present the case of a 54-year-old patient with metastatic GIST at diagnosis and the emergence of paraneoplastic manifestations during follow-up.

摘要

胃肠道间质瘤(GISTs)是胃肠道最常见的间叶组织肿瘤。酪氨酸激酶受体KIT或血小板衍生生长因子受体α(PDGFRA)的激活突变是导致该疾病的主要机制。患者通常表现为非特异性症状,而一些肿瘤是偶然发现的,诊断时可能已发生转移。侵袭性GISTs具有特定的转移模式,可转移至肝脏或整个腹部,或两者皆有。虽然GISTs很少出现全身性或孤立性副肿瘤反应,但文献中已有少数病例报道。我们报告了一例54岁诊断为转移性GIST的患者,在随访期间出现了副肿瘤表现。

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