Epithelioid malignant peripheral nerve sheath tumor of the bladder and concomitant urothelial carcinoma: A case report  

作　　者：Sami Berk Ozden Muhammed Fatih Simsekoglu Ipek Sertbudak Cetin Demirdag Iclal Gurses 

机构地区：[1]Istanbul University-Cerrahpasa,Cerrahpasa School of Medicine Department of Urology,Istanbul 34100,Fatih,Turkey [2]Istanbul University-Cerrahpasa,Cerrahpasa School of Medicine Department of Pathology,Istanbul 34100,Fatih,Turkey

出　　处：《World Journal of Clinical Cases》2024年第3期551-559,共9页世界临床病例杂志

摘　　要：BACKGROUND Epithelioid malignant peripheral nerve sheath tumor(EMPNST)of the bladder is a rare entity with devastating features.These tumors are thought to originate from malignant transformation of pre-existing schwannomas of pelvic autonomic nerve plexuses,and unlike the conventional malignant peripheral nerve sheath tumor(MPNST),are not associated with neurofibromatosis.The tumor has dis-tinctive morphological,immunohistochemical and molecular features.Addi-tionally,it tends to be more aggressive and have a higher mortality.This is the first case that presents with a synchronous urothelial carcinoma of the bladder and the epithelioid variant of MPNST in the literature.It’s also the second re-ported case of EMPNST originating from the bladder wall.CASE SUMMARY In this case report,we present the detailed clinical course of a 71-year-old patient with EMPNST of the bladder alongside a literature review.CONCLUSION During the management of EMPNST cases,offering aggressive treatment moda-lities to the patient,such as radical cystectomy,is appropriate for the best chance to contain the disease,regardless of the tumor stage and the extent of local disease at initial diagnosis.

关 键 词：EPITHELIOID Peripheral nerve sheath tumor BLADDER Case report UROTHELIAL CYSTOPROSTATECTOMY 

分 类 号：R737.14[医药卫生—肿瘤]