Clinicopathological and molecular biological characteristics of Wilms tumor with overlapping morphologically features of metanephric adenoma
ZHANG Dong-mei
HAN Jing
XU Yue
WANG Wei-wei
LUO Zhen-yu
LI Xin-qiang
WANG Xiao-juan
WEI Jian-guo
Abstract：Objective To explore the clinicopathological features,diagnosis,differential diagnosis and prognosis of Wilms tumor(WT)with overlapping morphologically features of metanephric adenoma(MA).Methods The clinicopathological features,immunophenotype and molecular pathological features of WT with overlapping morphologically features of MA were reviewed.Results The patient was a 16-year-old female.Histological examination revealed that the tumor was triphasic but spindled cells-predominant and contained differentiated areas that overlapped morphologically with MA.Focal anaplasia was observed.Immunohistochemical analysis revealed that the tumor cells diffusely expressed WT-1.The Ki67 proliferative index was significantly higher in the WT areas than that in the MA-like areas.Fluorescence PCR revealed the BRAF V600E mutation.31 cases(including our case)of WTs with overlapping morphologically features of MA were reported,of which 28 cases(87.10%)were adults.Except for our case,all other tumors were epithelial-predominant WTs.BRAF mutations were identified in 25 cases(83.3%).The follow-up data of 14 patients were analyzed(range,3-145 months),and all the patients survived.Conclusion WTs with overlapping morphologically features of MA are mostly epithelial-predominant,and harbor BRAF mutations.However,this subtype of tumors is not strictly confined to epithelial-predominant WTs.Our case is the first WT with overlapping morphologically features of MA which exhibit focal anaplasia,expanding the morphological spectrum of this subtype of tumors.These tumours may have a favorable prognosis and can be effectively treated with targeted therapy.
Keywords：Wilms tumorMetanephric adenomaBRAF V600ERenal tumor
Publication Date：2024-10-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 939-943 )