琥珀酸脱氢酶缺陷型胃肠道间质瘤临床病理分析(1)
【摘要】 目的：探讨琥珀酸脫氢酶(Succinate dehydrogenase，SDH)缺陷型胃肠道间质瘤(GIST)的临床病理特征、诊断、治疗以及独立分型的意义。方法：应用免疫组织化学EnVision法检测SDH亚单位B(SDHB)在110例胃GIST患者肿瘤组织中的表达情况，对其中SDHB缺陷型GIST的临床特点、组织病理学特点及免疫表型特点进行分析并随访。结果：110例GIST中6例SDHB蛋白表达阴性(SDHB缺陷)，占5.45%(6/110)，其中男2例，女4例；年龄14～65岁(平均35.8岁)；均发生于胃，胃窦3例，胃小弯侧2例，胃底1例；大体检查：肿瘤呈多结节状、分叶状，大小3～11 cm，切面实性，部分囊性变；镜下：瘤细胞以上皮样细胞为主，部分可混杂梭形细胞，其中上皮样细胞型4例，混合细胞型2例；2例可见脉管内瘤栓；6例患者均获得随访结果，皆生存；免疫组化：CD117(+)、DOG1(+)、CD34(+)、S-100(-)、SMA(-)、CK(-)、Ki-67阳性指数2%～10%、SDHB(-)。结论：SDH缺陷型GIST是一种特殊类型的GIST，具有独特的临床病理特征，其多累及儿童和青年人，女性多见，呈多结节状生长，镜下以上皮样细胞为主，常可见脉管内瘤栓、常发生淋巴结及肝脏转移，生物学行为惰性，治疗不同于经典的GIST，均为野生型病例且预后较好，免疫组织化学SDHB检测有助于将之从普通型GIST中检出。
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【关键词】 琥珀酸脱氢酶； 胃肠道间质瘤； 上皮样细胞
【Abstract】 Objective：To investigate the clinicopathological characteristics，diagnosis，treatment and independent typing of Succinate dehydrogenase(SDH) deficiency gastrointestinal stromal tumor(GIST).Method：Immunohistochemical EnVision method was used to detect the expression of SDH subunit B(SDHB) in 110 cases of gastric GIST，the clinical features，histopathological features and immunophenotype characteristics of SDHB deficient GIST were analyzed and followed up.Result：Of the 110 cases of GIST，6 cases(5.45%) were SDHB protein negative(SDHB defect)，of which 2 cases were male and 4 cases were female，the age was 14-65 years old(mean 35.8 years old)，all cases occurred in the stomach，antrum of 3 cases，small curved side of 2 cases，fundus of stomach in 1 case.Gross examination：the tumor was multi-nodular，lobulated，3-11 cm in size，solid on section and partly cystic.Microscopic examination：tumor cells were mainly composed of epithelioid cells and some were mixed with spindle cells，included epithelioid cells in 4 cases and mixed cells in 2 cases.2 cases showed intravascular tumor thrombus.All patients were followed up and survived.Immunohistochemistry：CD117(+)，DOG1(+)，CD34(+)，S-100(-)，SMA(-)，CK(-)，the positive Ki-67 index was 2%-10%，SDHB(-).Conclusion：SDH deficient GIST is a special type of GIST with unique clinicopathological features，the involvement of children and young people，women，a multinodular growth，with epithelioid in appearance，often visible intravascular tumor embolus，often occur in lymph node and liver metastasis，inert biological behavior，the treatment of GIST，which is different from the classic，is a wild type case with better prognosis，immuno histochemical SDHB detection helps to detect it from the common GIST.
【Key words】 Succinic dehydrogenase； Gastrointestinal stromal tumor； Epithelioid cells, 百拇医药(马明 冯瑶 肖虹)
, http://www.100md.com
【关键词】 琥珀酸脱氢酶； 胃肠道间质瘤； 上皮样细胞
【Abstract】 Objective：To investigate the clinicopathological characteristics，diagnosis，treatment and independent typing of Succinate dehydrogenase(SDH) deficiency gastrointestinal stromal tumor(GIST).Method：Immunohistochemical EnVision method was used to detect the expression of SDH subunit B(SDHB) in 110 cases of gastric GIST，the clinical features，histopathological features and immunophenotype characteristics of SDHB deficient GIST were analyzed and followed up.Result：Of the 110 cases of GIST，6 cases(5.45%) were SDHB protein negative(SDHB defect)，of which 2 cases were male and 4 cases were female，the age was 14-65 years old(mean 35.8 years old)，all cases occurred in the stomach，antrum of 3 cases，small curved side of 2 cases，fundus of stomach in 1 case.Gross examination：the tumor was multi-nodular，lobulated，3-11 cm in size，solid on section and partly cystic.Microscopic examination：tumor cells were mainly composed of epithelioid cells and some were mixed with spindle cells，included epithelioid cells in 4 cases and mixed cells in 2 cases.2 cases showed intravascular tumor thrombus.All patients were followed up and survived.Immunohistochemistry：CD117(+)，DOG1(+)，CD34(+)，S-100(-)，SMA(-)，CK(-)，the positive Ki-67 index was 2%-10%，SDHB(-).Conclusion：SDH deficient GIST is a special type of GIST with unique clinicopathological features，the involvement of children and young people，women，a multinodular growth，with epithelioid in appearance，often visible intravascular tumor embolus，often occur in lymph node and liver metastasis，inert biological behavior，the treatment of GIST，which is different from the classic，is a wild type case with better prognosis，immuno histochemical SDHB detection helps to detect it from the common GIST.
【Key words】 Succinic dehydrogenase； Gastrointestinal stromal tumor； Epithelioid cells, 百拇医药(马明 冯瑶 肖虹)
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