伴有极向反转的乳头状肾肿瘤1例并文献复习  

Papillary renal neoplasm with reverse polarity:A case report and literature review

作　　者：常悦 刘霞 施琳 贾永峰[2,3] CHANG Yue;LIU Xia;SHI Lin;JIA Yongfeng(First School of Clinical Medicine,Inner Mongolia Medical University,Huhhot 010110;School of Basic Medical Sciences,Inner Mongolia Medical University,Huhhot 010110;Department of Pathology,Inner Mongolia Medical University Hospital,Huhhot 010059,China)

机构地区：[1]内蒙古医科大学第一临床医学院,呼和浩特010110 [2]内蒙古医科大学基础医学院,呼和浩特010110 [3]内蒙古医科大学附属医院病理科,呼和浩特010059

出　　处：《临床与病理杂志》2025年第1期110-115,共6页Journal of Clinical and Pathological Research

摘　　要：伴有极向反转的乳头状肾肿瘤(papillary renal neoplasm with reverse polarity,PRNRP)是一种罕见的肾肿瘤,具有特殊的组织学形态,患者多无明显特殊症状,多于体检或因其他疾病就诊时发现,容易误诊或漏诊。内蒙古医科大学附属医院2023年4月24日收治1例罕见的PRNRP患者,患者体检发现右肾肿物,行右肾部分切除术,肿物体积约为4 cm×3 cm×1 cm,大体囊实性,镜下见肿瘤细胞主要为伴有纤维血管轴心的乳头状或管状结构,被覆单层的立方或柱状上皮细胞,细胞形态温和,大小基本一致,胞质嗜酸性,细胞核规律分布于远离基底膜的胞质顶端。免疫组织化学染色肿瘤细胞表达细胞角蛋白7、配对盒基因8、上皮膜抗原、GATA结合蛋白3,部分表达波形蛋白,不表达CD10、中枢神经特异蛋白S-100及转录因子结合免疫球蛋白重链μ增强子3,Ki-67增殖指数小于1%。术后随访1年,无复发及转移。由于发病罕见,目前对其报道较少,需要积累更多病例来揭示其生物学行为。本病例报道旨在提高临床医师对伴有极向反转的乳头状肾肿瘤诊疗的认识,避免误诊导致过度治疗及漏诊的发生。Papillary renal neoplasm with reverse polarity(PRNRP)is a rare subtype of renal tumor characterized by distinct histological features.Patients typically present without specific symptoms,and the tumor is often detected incidentally during physical examinations or evaluation for unrelated conditions,which makes misdiagnosis or missed diagnosis common.On April 24,2023,the Inner Mongolia Medical University Affiliated Hospital admitted a rare case of PRNRP.The patient was found to have a right renal mass during a routine physical examination and underwent partial nephrectomy.The tumor measured approximately 4 cm×3 cm×1 cm and appeared cystic-solid on gross examination.Microscopically,the tumor exhibited papillary or tubular structures with fibrovascular cores lined by a single layer of cuboidal to columnar epithelial cells.The tumor cells displayed mild cytologic atypia,uniform size,eosinophilic cytoplasm,and nuclei regularly positioned at the apical side away from the basement membrane.Immunohistochemical staining revealed positive expression of cytokeratin 7,paired box gene 8,epithelial membrane antigen,and endothelial transcription factor 3,with partial expression of vimentin.The tumor cells were negative for CD10,central nervous system specific protein S-100,and transcription factor binding to immunoglobulin heavy chain mu enhancer 3(TFE3),and showed a Ki-67 proliferation index of less than 1%.The patient was followed up for 1 year postoperatively with no evidence of recurrence or metastasis.Due to its rarity,reports on PRNRP remain limited,and more cases are needed to better understand its biological behavior.This case report aims to enhance clinical awareness of PRNRP,helping to avoid misdiagnosis,overtreatment,or missed diagnoses.

关 键 词：乳头状肾肿瘤 极向反转 KRAS 鉴别诊断 免疫组织化学 

分 类 号：R737.11[医药卫生—肿瘤]