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目的:探讨原发性肾上腺血管周上皮样细胞瘤(PEComa)的临床病理特点及诊断治疗方法。方法:报告2014年11月中国人民解放军总医院泌尿外科诊治原发性肾上腺PEComa 1例患者的临床特征、影像学特点、病理学结果、治疗及随访情况,并结合文献进行分析。结果:文献报道的肾上腺PEComa非常罕见,该病术前多无特异临床症状,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结果,治疗首选手术切除,术后须长期随访。结论:原发性肾上腺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 adrenal glandular epithelioid cell tumor (PEComa). METHODS: The clinical features, imaging features, pathological findings, treatment and follow-up of 1 patient with primary adrenal PEComa diagnosed and treated in the Department of Urology, Chinese PLA General Hospital were reported in November 2014 and analyzed in combination with the literature. Results: The adrenal PEComa reported in the literature is very rare. There are no specific clinical symptoms before the operation. It is easily found by imaging method, but it is difficult to diagnose. The final diagnosis relies on pathological findings and immunohistochemical results, and the treatment is the first choice of surgical resection. Long-term follow-up. Conclusions: Primary adrenal PEComa is a very rare mesenchymal tumor that is diagnosed by pathological methods. Its biological behavior is not clear, it is recommended long-term follow-up.