低度恶性潜能多房囊性肾肿瘤32例临床病理分析

Multilocular cystic renal neoplasm of low malignant potential:a clinicopathological analysis of 32 cases

目的分析低度恶性潜能多房囊性肾肿瘤(MCRNLMP)的临床病理学特征,提高对该肿瘤的认识。方法回顾性分析32例MCRNLMP的临床资料、组织学形态、免疫组化,并进行相关文献复习,探讨其临床病理特征。结果32例MCRNLMP中,男女比为23∶9,年龄29~73岁,平均49.9岁;左侧肾15例,右侧17例,多为体检发现肾占位。镜下多数囊腔内衬单层细胞,细胞核WHO/ISUP 1级或2级,部分间隔内可见小簇状透明细胞,但并非膨胀性生长。免疫组化示32例瘤细胞均CK(AE1/AE3)、vimentin、碳酸酐酶IX(CAIX)、PAX-2和PAX-8(+),28例CD10(+);CD68、TFE3(-),Ki-67增殖指数1~3%。随访3~78个月,均无复发及转移。结论MCRNLMP具有低度恶性潜能,预后极好。组织学上肿物以囊性成分为主,囊壁上皮部分为透明细胞,须与多种肾囊性病变鉴别。术前确诊困难,主要依靠术后病理明确诊断,同时也需结合临床及影像学资料。

Objective To improve clinicians and pathologists’understanding of multilocular cystic clear cell neoplasm of low malignant potential(MCCNLMP).Methods The clinical data,histology and immunohistochemical phenotype were retrospectively analyzed in 32 cases of MCCNLMP,and its clinicopathological characteristics reviewed.Results In the 32 cases,it occurred in 23 men and 9 women(the rate was 23∶9),aged 29 to 73 years(average 49.9 years).There were 15 cases in left kidney and 17 case in the right,primarily presenting masses as physical examination.Under microscope,most cysts were lined by single tumor cells,and the nuclei were WHO/ISUP grade 1 or 2.Clear cells were present within the fibrous septa without expansile growth.Immunohistochemically,tumor cells expressed for CK(AE1/AE3),arbonic anhydrase IX(CAIX),PAX-2,and PAX-8,while 28 cases were positive for CD10.All cases were negative for CD68 and TFE3,and Ki-67 proliferation index was 1~3%.With following-up 3 to 78 months,there was no recurrence or metastasis.Conclusion MCCNLMP is a low malignant potential tumor,and its has good prognosis.Pathological features are mainly cystic components with clear cells,and this tumor must be differentiated from other renal lessons.It is difficult to make correct diagnosis before surgical resection.The diagnosis relays on postoperative pathological examination,combined with clinical and imaging data.