原发前列腺部位胃肠外间质瘤4例报告并文献复习

Primary extra-gastrointestinal stromal tumor of the prostate:4 cases report and literature review

目的提高对前列腺部位胃肠外间质瘤(EGISTs)病因、临床特点、诊断及治疗的认识。方法我院2010年至2014年收治4例发生于前列腺部位的EGISTs患者,术前诊断为前列腺肿瘤、直肠肿物,均行盆腔肿物切除术。报告其中1例临床资料,并行文献复习。结果患者因排便困难伴血便3月入院,直肠指诊发现直肠前壁黏膜下有一质韧肿物,考虑为直肠肿物。盆腔的B超及MRI检查均提示肿物可能来源于前列腺。盆腔肿物探查术后病理结果提示为(前列腺)间叶组织肿瘤,梭形细胞为主,增生活跃,诊断为恶性EGISTs;免疫组化检查CD117、CD34均阳性。结论前列腺部位EGISTs发病与C-kit和PDGFRA基因状态有着密切联系,临床表现有会阴部酸疼不适、尿频、尿急、排尿困难、肉眼血尿、排便困难、血便,术前确诊需结合影像学检查和肿瘤穿刺病理免疫组化检测,治疗以手术为主,无手术适应证的患者可行药物辅助靶向治疗。

Objective To improve the understanding of the etiology,clinical characteristics,diagnosis and treatment of extra-gastrointestinal stromal tumors of the prostate.Methods Four cases of EGISTs of the prostate were reported and the related literatures were reviewed.Results The patients clinical symptoms included difficult defecation and bloody stool.One strong but pliable tumor was found under mucosa of posterior wall of rectum by digital rectal examination,which was identified as rectal neoplasms.B-ultrasonic examination and MRI of the pelvic suggested that the tumor probably was derived from the prostate.The postoperative pathological results showed mesenchymal tissue tumor from the prostate.Immunohistochemical findings revealed that the neoplastic cells were positive for CD34 and CD117.Conclusion Primary extra-gastrointestinal stromal tumor of the prostate has intimate connection with the status of C-kit and PDGFRA.Its clinical features may include perineal pain,frequent urination,urgency,macroscopic hematuria,difficult defecation and bloody stool.Preoperative diagnosis should be combined with imaging examination and biopsy in immunohistochemical detection.Surgeries are the main mode of treatment.Therapy targeting of tyrosine protein kinase should be used for the unresectable cases.