间变性淋巴瘤激酶基因重排在肺外炎性肌纤维母细胞瘤中的表达及其临床意义

Expression of ALK protein in extra-pulmonary inflammatory myofibroblastic tumor and its clinical significance

目的探讨间变性淋巴瘤激酶（ALK）蛋白在肺外炎性肌纤维母细胞瘤（IMT）中的表达情况，并分析其与临床复发之间的关系。方法回顾性分析2008年1月1日至2012年12月31日期间在我院外科诊治并经病理诊断的肺外IMT14例，分别采用免疫组织化学技术Envision法染色和双色原位杂交技术，了解本组患儿标本中ALK表达情况，分析ALK阳性表达与局部复发之间的关系，确定ALK表达的临床意义。结果本组14例中，男10例，女4例；年龄2个月～13岁；肿瘤最大30cmX25cm×15cm，来源于腹膜后；肿瘤最小0．8cm×0．8cm×0．5cm，来源于膝部。免疫组织化学结果：ALK（＋）11例，同时行FISH检测，其中有2例发现有ALK基因重排，都是局部复发病例。再次手术切除复发病变，其免疫组织化学ALK仍为阳性表达。术后辅以化疗，随访2年未见复发。结论IMT发病率较低，手术切除是治疗IMT的可靠方式；ALK蛋白表达可以证实IMT诊断，而ALK基因重排与肿瘤局部复发可能相关。

Objective To explore the anaplastic l^y[nphoma kinase （ALK） expression in extra- pulmonary inflammatory myofibroblastic tumor （IMT） and analyze its relationship with clinical local recurrence. Methods Retrospective clinical data reviews were performed for 14 cases of inflammatory myofibroblastic tumor at Tianjin Children＇s Hospital from January 1,2008 to December 31,2012. Immunohistochemical technology Envision staining and dual-color fluorescencein in situ hybridization （FISH） were employed to determine the ALK expression in whole specimens and analyze the relationship between clinical expression of ALK positive cells and local tumor recurrence. Results There were 10 males and 4 females with an age range of 2 months to 13 years. The largest diameter of retroperitoneal tumor was 30 cm × 25 cm × 15 cm and a minimal diameter of knew at 0. 8 cm × 0. 8 cm × 0. 5 cm The results of immunohistochemistry staining showed 11 eases of ALK （＋）. A further detection of FISH revealed 2 cases of ALK gene rearrangement as local recurrence. After resection of recurrent lesion, immunohistochemical staining ALK expression was still positive. Postoperative chemotherapy was administered. There was no recurrence during a follow-up of 2 years. Conclusions The incidence rate of IMT is low and its operation is reliable. And the expression of ALK protein may confirm its diagnosis and local tumor recurrence is probably related with ALK gene rearrangement.