Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 26, 2024; 12(3): 551-559
Published online Jan 26, 2024. doi: 10.12998/wjcc.v12.i3.551
Epithelioid malignant peripheral nerve sheath tumor of the bladder and concomitant urothelial carcinoma: A case report
Sami Berk Ozden, Muhammed Fatih Simsekoglu, Ipek Sertbudak, Cetin Demirdag, Iclal Gurses
Sami Berk Ozden, Muhammed Fatih Simsekoglu, Cetin Demirdag, Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Urology, Istanbul 34100, Fatih, Turkey
Ipek Sertbudak, Iclal Gurses, Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Pathology, Istanbul 34100, Fatih, Turkey
Author contributions: Demirdag C was head surgeon; Gurses I was head pathologist; Ozden SB wrote the manuscript; Sertbudak I constructed the study design; Şimşekoğlu MF revised the text and collected patient data.
Informed consent statement: Informed consent was obtained from all participants included in the study.
Conflict-of-interest statement: All the authors declare that they have no conflict of interest.
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Corresponding author: Sami Berk Ozden, MD, Academic Research, Researcher, Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine Department of Urology, Cerrahpaşa Tıp Fakültesi Yerleşkesi Kocamustafapaşa Caddesi No: 53 Cerrahpaşa 34098 Fatih/İstanbul, Istanbul 34100, Fatih, Turkey. s.berkozden@gmail.com
Received: April 28, 2023
Peer-review started: April 28, 2023
First decision: July 27, 2023
Revised: August 8, 2023
Accepted: August 17, 2023
Article in press: August 17, 2023
Published online: January 26, 2024
Processing time: 264 Days and 13.3 Hours
Abstract
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.

Keywords: Epithelioid; Peripheral nerve sheath tumor; Bladder; Case report; Urothelial; Cystoprostatectomy

Core Tip: In this case report, we describe a patient diagnosed with epithelioid variant of malignant peripheral nerve sheath tumor of the bladder wall after thorough pathologic examination. The case is noteworthy, as it is the second reported case that provides information regarding the patient’s prognosis.