伴KRAS/NRAS突变型子宫颈中肾管腺癌2例临床病理分析

Cervical mesonephric adenocarcinoma with KRAS/NRAS mutation:a clinicopathological analysis of two cases

目的探讨子宫颈中肾管腺癌(mesonephric adenocarcinoma,MNA)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析2例子宫颈MNA的临床资料、病理学形态及免疫表型,并复习相关文献。结果2例患者年龄分别为51、77岁,临床均表现为阴道不规则出血、子宫颈赘生物;术前活检诊断及会诊结果为子宫颈低分化癌、鳞状细胞癌、癌肉瘤、间质肉瘤、滑膜肉瘤等;镜下肿瘤组织以实性巢状、条索状结构为主,肿瘤细胞圆形或梭形,局灶可见少量腺样结构,腺腔内可见嗜酸性分泌物;免疫表型:肿瘤细胞vimentin、CK(AE1/AE3)和PAX8均弥漫强(+),梭形细胞CD10弥漫强(+),EMA、p16和p53灶(+),ER、PR、CEA均(-),Ki-67增殖指数20%~60%;基因检测结果显示:2例均存在KRAS基因2号外显子p.G12A突变,例2还同时存在NRAS基因3号外显子p.A59D突变。结论子宫颈MNA是来源于中肾管残件的罕见肿瘤,生物学行为与其他类型子宫颈腺癌及肉瘤相比更加惰性,但其病理学形态多样,尤以梭形细胞成分为主时,极易误诊。认识子宫颈MNA不同组织学形态,寻找特征性诊断线索,对该肿瘤的正确诊断有重要临床意义。KRAS/NRAS分子检测为临床尝试靶向治疗提供依据。

Purpose To investigate the clinicopathological features,diagnosis and differential diagnosis of mesonephric adenocarcinoma(MNA)in cervical.Methods The clinical data,histological morphology and expression of immunohistochemical markers in 2 patients with cervical MNA were analyzed and related literatures were reviewed.Results The ages of the patients were 51 years and 77 years respectively.The clinical manifestations were irregular vaginal bleeding and cervical neoplasms.Preoperative biopsy diagnosis and consultation results were:poorly differentiated cervical cancer,squamous cell carcinoma,carcinosarcoma,interstitial sarcoma,synovial sarcoma etc.Under microscope,the tumor tissue was mainly solid nested and strip-like structure,tumor cells were round or fusiform,and a small amount of adenoid structure was seen in the focal solid nest,with eosinophilic secretion in the glandular lumen,immunophenotyping showed that tumor cells were positive for vimentin,CK(AE1/AE3)and PAX8,fusiform cells are strongly positive for CD10.Uneven expression of EMA,p16 and p53 was noted but negative for ER,PR and CEA,Ki-67 index was 20%-60%.The results of gene detection showed that there were mutations in exon 2 of KRAS gene in both cases,and case 2 also had the p.A59D mutation in exon 3 of NRAS gene.Conclusion Cervical MNA is a rare tumor originated from the wolffian duct.The biological behavior is more inert than other types of cervical adenocarcinoma and sarcoma,but its pathological morphology is diverse,especially when fusiform cell components are the main components,it is easily misdiagnosed.Understanding the different histological morphology of cervical MNA,looking for characteristic diagnostic clues,has important clinical significance for the correct diagnosis of this tumor.KRAS/NRAS molecular detection provides a basis for clinical attempts to target therapy.