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

被引:0
作者
Ozden, Sami Berk [1 ,3 ]
Simsekoglu, Muhammed Fatih [1 ]
Sertbudak, Ipek [2 ]
Demirdag, Cetin [1 ]
Gurses, Iclal [2 ]
机构
[1] Istanbul Univ Cerrahpasa, Cerrahpasa Sch Med, Dept Urol, TR-34100 Istanbul, Turkiye
[2] Istanbul Univ Cerrahpasa, Cerrahpasa Sch Med, Dept Pathol, TR-34100 Istanbul, Turkiye
[3] Istanbul Univ Cerrahpasa, Cerrahpasa Sch Med, Dept Urol, Cerrahpasa Tip Fak Yerleskesi Kocamustafapasa Cadd, TR-34100 Istanbul, Fatih, Turkiye
关键词
Epithelioid; Peripheral nerve sheath tumor; Bladder; Case report; Urothelial; Cystoprostatectomy; SOFT-TISSUE; SARCOMA;
D O I
10.12998/wjcc.v12.i3.551
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
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 distinctive morphological, immunohistochemical and molecular features. Additionally, 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 reported 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 modalities 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.
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页数:10
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