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Lancet Oncol. 2002 Nov;3(11):655-64. doi: 10.1016/s1470-2045(02)00899-9.
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Germline mutation in the juxtamembrane domain of the kit gene in a family with gastrointestinal stromal tumors and urticaria pigmentosa.一个患有胃肠道间质瘤和色素性荨麻疹的家族中,kit基因近膜结构域的种系突变。
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Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis.胃肠道间质瘤——定义、临床、组织学、免疫组化及分子遗传学特征与鉴别诊断
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Familial gastrointestinal stromal tumor with hyperpigmentation: association with a germline mutation of the c-kit gene.伴有色素沉着的家族性胃肠道间质瘤:与c-kit基因种系突变相关
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Ann Surg Oncol. 2000 Oct;7(9):705-12. doi: 10.1007/s10434-000-0705-6.
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Ann Surg. 2000 Jan;231(1):51-8. doi: 10.1097/00000658-200001000-00008.
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Gastrointestinal stromal tumors: recent advances in understanding of their biology.胃肠道间质瘤:对其生物学认识的最新进展
Hum Pathol. 1999 Oct;30(10):1213-20. doi: 10.1016/s0046-8177(99)90040-0.
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Effect of c-kit mutation on prognosis of gastrointestinal stromal tumors.c-kit突变对胃肠道间质瘤预后的影响。
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Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence.胃间质肉瘤、肺软骨瘤和肾上腺外副神经节瘤(卡尼三联征):自然病史、肾上腺皮质成分及可能的家族性发病情况。
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香港一组中国患者的胃肠道间质瘤

Gastrointestinal stromal tumors in a cohort of Chinese patients in Hong Kong.

作者信息

Chan Kam Hoi, Chan Chun Wing, Chow Wai Hung, Kwan Wai Keung, Kong Chi Kwan, Mak Ka Fung, Leung Miu Yi, Lau Lin Kiu

机构信息

Department of Medicine, Yan Chai Hospital, Tsuen Wan, Hong Kong SAR, China.

出版信息

World J Gastroenterol. 2006 Apr 14;12(14):2223-8. doi: 10.3748/wjg.v12.i14.2223.

DOI:10.3748/wjg.v12.i14.2223
PMID:16610025
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4087650/
Abstract

AIM

To investigate the prevalence and clinical pattern of gastrointestinal stromal tumors (GISTs) in Hong Kong Chinese, and to assess the impact of introduction of CD117 on the disease incidence.

METHODS

From the database of the Department of Pathology of Yan Chai Hospital, 47 patients, with GISTs from September 1995 to December 2003 were included in this study. Ten GISTs were diagnosed before the introduction of CD117. The clinical features, tumor characteristics, and treatment were analyzed. Factors predicting tumor related death or recurrence were studied with Cox proportional hazard model.

RESULTS

The patients included 26 males and 21 females, with a mean age of 66.6 years (SD 13.1, range 29-87 years). The estimated prevalence of GISTs was 13.4-15.6 per 100,000 people, with an annual incidence of 1.68-1.96 per 100,000 people. The annual incidence of GISTs before and after the introduction of CD117 was 1.1 per 100,000 people and 2.1 per 100,000 people respectively. Stomach (34 patients, 72.3%) was the most common location for the tumor, followed by the small intestine (8 patients, 17.0%), esophagus (2 patients, 4.3%), omentum (2 patients, 4.3%) and colon (1 patient, 2.1%). Thirty-one patients (66%) had complete tumor resection. Eleven out of 16 deaths (23%) were tumor-related. The median survival time was 26 mo. Five-year survival rate was 61.3%. The significant factors associated with tumor-related death or recurrence were incomplete resection, tumor size 5 cm or above, invasion to the adjacent organ or presence of metastasis.

CONCLUSION

The incidence of GIST in Hong Kong is comparable to that in the United States but lower than that in Finland. The true incidence of GISTs could be underestimated before the introduction of CD117. Incomplete resection, tumor size 5 cm or above, invasion to the adjacent organ or presence of metastasis are factors predicting tumor-related death or recurrence.

摘要

目的

调查香港华人胃肠道间质瘤(GISTs)的患病率和临床模式,并评估引入CD117对疾病发病率的影响。

方法

从仁济医院病理科数据库中纳入1995年9月至2003年12月期间的47例GIST患者。10例GIST在引入CD117之前被诊断。分析临床特征、肿瘤特征和治疗情况。采用Cox比例风险模型研究预测肿瘤相关死亡或复发的因素。

结果

患者包括26名男性和21名女性,平均年龄66.6岁(标准差13.1,范围29 - 87岁)。GIST的估计患病率为每10万人13.4 - 15.6例,年发病率为每10万人1.68 - 1.96例。引入CD117之前和之后GIST的年发病率分别为每10万人1.1例和每10万人2.1例。胃(34例患者,72.3%)是肿瘤最常见的部位,其次是小肠(8例患者,17.0%)、食管(2例患者,4.3%)、网膜(2例患者,4.3%)和结肠(1例患者,2.1%)。31例患者(66%)进行了肿瘤完整切除。16例死亡患者中有11例(23%)与肿瘤相关。中位生存时间为26个月。5年生存率为61.3%。与肿瘤相关死亡或复发相关的显著因素是切除不完全、肿瘤大小5厘米或以上、侵犯相邻器官或存在转移。

结论

香港GIST的发病率与美国相当,但低于芬兰。在引入CD117之前,GIST的真实发病率可能被低估。切除不完全、肿瘤大小5厘米或以上、侵犯相邻器官或存在转移是预测肿瘤相关死亡或复发的因素。