复发性腹膜后非胃肠道间质瘤伴横纹肌肉瘤和软骨肉瘤分化：1例罕见病例及文献复习
Recurrent retroperitoneal extra-GIST with rhabdomyosarcomatous and chondrosarcomatous differentiations: a rare case and literature review.
作者信息
Zhu Pengcheng, Fei Yan, Wang Yue'e, Ao Qilin, Wang Guoping
机构信息
Institute of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology Wuhan 430030, China.
Department of Oncology, Zhongshan Hospital of Hubei Province Wuhan 430000, China.
出版信息
Int J Clin Exp Pathol. 2015 Aug 1;8(8):9655-61. eCollection 2015.
Selective tyrosine kinase inhibitor (TKI) targeting KIT and PDGFRA is the frontline therapy for metastatic and unresectable GIST patients. Some initially responsive patients experience tumor progress because of secondary drug resistance, and some cases can develop heterogeneous differentiation. Here we report a rare case of recurrent retroperitoneal extra-GIST with rhabdomyosarcomatous and chondrosarcomatous differentiation with TKI therapy after surgical tumorectomy. Histology, immunohistochemistry, and mutational analysis were performed on primary and recurrent samples. The current case represents the first report of a recurrent retroperitoneal extra-GIST harboring mixed morphologic phenotypes of rhabdomyosarcoma and chondrosarsoma after TKI treatment. The dual differentiation can represent diagnostic pitfall.
针对KIT和PDGFRA的选择性酪氨酸激酶抑制剂（TKI）是转移性和不可切除胃肠道间质瘤（GIST）患者的一线治疗方法。一些最初有反应的患者会因继发性耐药而出现肿瘤进展，还有一些病例会发生异质性分化。在此，我们报告一例罕见的复发性腹膜后GIST外肿瘤病例，该肿瘤在手术切除肿瘤后接受TKI治疗，具有横纹肌肉瘤和软骨肉瘤分化。对原发和复发样本进行了组织学、免疫组织化学和突变分析。本病例是TKI治疗后出现横纹肌肉瘤和软骨肉瘤混合形态学表型的复发性腹膜后GIST外肿瘤的首例报告。这种双重分化可能会造成诊断陷阱。
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