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目的探讨原发性腹膜后血管周上皮样细胞瘤(PEComa)的临床病理特点及诊断治疗。方法报告2009年4月北京协和医院基本外科诊治的位于盆腔的原发性腹膜后PEComa1例病人的症状、影像、病理、治疗及随访情况,并结合文献进行分析。结果术前无特异临床症状,为查体发现。术前容易通过影像学手段发现但难以确诊。最终诊断依靠病理学表现和免疫组化。文献报道的原发性腹膜后PEComa非常罕见。结论原发性腹膜后PEComa是一种非常罕见的间叶组织肿瘤,依靠病理学方法诊断。其生物学行为不明确,建议术后长期随访。
Objective To investigate the clinicopathological characteristics and diagnosis and treatment of primary retroperitoneal pericytes. Methods The symptoms, imaging, pathology, treatment and follow-up of one case of primary retroperitoneal PEComa in pelvic cavity diagnosed and treated in Peking Union Medical College Hospital in April 2009 were reported and analyzed in combination with the literature. Results Preoperative no specific clinical symptoms, found for the examination. Preoperative easy to find by imaging means but difficult to diagnose. The final diagnosis depends on pathology and immunohistochemistry. Reported in the literature of primary retroperitoneal PEComa is very rare. Conclusions Primary retroperitoneal PEComa is a very rare mesenchymal tumor that is diagnosed by pathology. Its biological behavior is not clear, it is recommended long-term follow-up.