【摘要】 目的探讨女性生殖系统原发血管周上皮样细胞肿瘤(Perivascular Epithelioid Cell tumor,PEComa)的临床病理、免疫组化特点及鉴别诊断。方法收集四川大学华西第二医院2008-01—2017-12诊断的女性生殖系统原发PEComa共4例,对其进行病理组织形态学观察和免疫组化染色检测,并复习国内外相关文献。结果患者年龄从18～50岁(平均年龄34.75岁)。2例发生于宫体肌层,1例发生于宫腔,1例发生于宫颈。其中2例PEComa分别合并了输卵管异位妊娠和宫内妊娠。发生于宫颈的PEComa为恶性PEComa,虽然肿瘤细胞仅有轻度异型性,但肿瘤在宫颈间质内呈广泛舌状浸润,同时转移至左卵巢和左、右盆腔淋巴结。所有病例中肿瘤细胞均表达黑色素标记HMB45和Melan-A,其中2例病例还同时表达肌源性标记desmin。1例宫颈恶性PEComa病人术后27个月因病死亡,另3例病人均存活且无复发转移。结论女性生殖系统原发PEComa易带来病理诊断上的困难,组织形态特点结合免疫组织化学检测有助于正确鉴别诊断。更多还原

【Abstract】 Objective To study the clinicopathologic and immunohistochemical features and differentiatial diagnosis of the perivascular epithelioid cell tumor(PEComa) originated from the female reproductive system. Methods The samples of 4 cases of primary PEComa of the female reproductive system, collected from West China Second Hospital of Sichuan University from Jan. 2008 to Dec. 2017, were studied by means of clinical and follow-up data analysis, light microscopy and immunohistochemistry, and the related literatures were reviewed. Results The age of the 4 patients ranged from 18 to 50 years(mean 34.75 years). Our data included 2 cases of PEComa of the uterine corpus, 1 case of PEComa of the uterine cavity and 1 case of PEComa of the uterine cervix, in which 2 cases were combined with ectopic pregnancy and intrauterine pregnancy, respectively. Although tumor cells were mildly atypical, this case of PEComa originated from uterine cervix was malignant, which extensively permeated the myometrium of the cervix with "tongue-like" growth pattern and metastasized to left ovary and bilateral pelvic lymph node. Strong HMB45 and Melan-A immunopositivity was found in all cases; at the same time, 50% cases stained positive for desmin. 1 patient diagnosed as cervical malignant PEComa was dead and the other 3 patients were alive without recurrence and metastasis during the follow-up. Conclusion PEComa of the female reproductive system posesses several diagnostic difficulties. The combination of morphological characteristics with immunohistochemical findings could be help to the correct differential diagnosis.更多还原