卵巢恶性PEComa 1例报道并文献复习  

Ovarian malignant PEComa:A case report and review of the literature

作　　者：蒋梦凡 钱金锋[2] JIANG Mengfan;QIAN Jinfeng(Hai'an Hosptital of Traditional Chinese Medicine,Jiangsu Nantong 226600,China;The First People's Hospital of Nantong,Jiangsu Nantong 226014,China)

机构地区：[1]海安市中医院,江苏南通226600 [2]南通市第一人民医院,江苏南通226014

出　　处：《现代肿瘤医学》2024年第17期3280-3285,共6页Journal of Modern Oncology

摘　　要：目的:探讨卵巢原发恶性血管周上皮样肿瘤(perivascular epithelioid cell tumor,PEComa)的临床病理特征、诊断、鉴别诊断、治疗及预后。方法:对1例卵巢原发恶性PEComa进行常规病理学和免疫组化学标记分析并文献复习。结果:肿瘤由上皮样至梭形细胞组成,弥漫片状生长,部分区域可见瘤细胞围绕在血管周围呈放射状排列,瘤细胞胞质透亮或淡嗜伊红色,可见色素,见坏死及核分裂象。免疫组化:HMB45(+),Melan-A(+),TFE3(+),Ki-67 (指数<10%)。结论:卵巢原发恶性PEComa是一种罕见的表达黑色素标记和平滑肌标记的一组间叶性肿瘤,临床症状不典型,组织学形态及免疫标志物在诊断及鉴别诊断具有重要价值,术后影像学随访非常重要,免疫治疗及放射治疗仍值得探索。Objective:To investigate the clinicopathological features,diagnosis,differential diagnosis,treatment and prognosis of primary malignant perivascular epithelioid cell tumor(PEComa) in ovary.Methods:One case of primary malignant PEComa of the ovary was analyzed by conventional pathology and immunohistochemical markers and literature review.Results:The tumor consisted of epithelioid to spindle-shaped cells,diffuse sheet-like growth,some areas of tumor cells were seen to surround the blood vessels in a radial arrangement,the tumor cell cytoplasm was translucent or pale eosinophilic red,pigmentation was seen,and necrosis and nuclear schizophrenia were seen.Immunohistochemistry:HMB45(+),Melan-A(+),TFE3(+),Ki-67(index <10%).Conclusion:Primary malignant PEComa of the ovary is a rare group of mesenchymal tumors expressing melanin markers and smooth muscle markers,with atypical clinical symptoms,histological morphology and immunomarkers are of great value in diagnosis and differential diagnosis,postoperative imaging follow-up is very important,and immunotherapy and radiation therapy are still worth exploring.

关 键 词：卵巢肿瘤 血管周上皮样肿瘤 病理诊断 鉴别诊断 

分 类 号：R737.31[医药卫生—肿瘤]