Plasmacytoid Variant of Urothelial Carcinoma: Poor Prognostic Variant with High Expression of CDH1 Mutation

Alisa Erck,Simon Chung,Jeanny B Aragon-Ching,Saeid Movahedi-Lankarani,Wenping Li

doi:10.3390/uro1010004

Alisa Erck, Simon Chung + Show 3 more

Open Access

https://doi.org/10.3390/uro1010004

Copy DOI

Journal: Uro	Publication Date: Mar 4, 2021
Citations: 3	License type: CC BY 4.0

Affiliation: University of Virginia, Inova Fairfax Hospital

Abstract

Plasmacytoid variant of urothelial carcinoma is a rare subtype of urothelial carcinoma that has poor prognosis. We describe two cases of patients with the plasmacytoid variant of urothelial carcinoma (PVUC) who had initial response to neoadjuvant chemotherapy followed by radical cystoprostatectomy and lymph node dissection but presented with early relapse and disease progression manifesting with intestinal obstruction and peritoneal carcinomatosis. Tumor genomic sequencing revealed mutations and alterations in ARID1A, CDH1, PIK3CA, RB1 loss, and TERT promoter, as well as tumor mutational burden of 10 Muts/Mb treated with pembrolizumab with a minimal response. A further review of the literature regarding this rare variant is discussed here.

Highlights

The plasmacytoid variant of urothelial carcinoma (PVUC) makes up a rare subtype of urothelial cancers
It is predominantly observed in males above 55 years of age and is often associated with higher lymph node involvement, locally advanced tumors, and muscle-invasive disease, and it is more likely to be diagnosed at higher stages compared to pure urothelial carcinomas (UCs) [5,6]
Tumor cells were initially found during the transurethral resection of bladder tumor (TURBT) of the bladder, and while successful surgery was done with cystoprostatectomy and lymph node dissection with ileal diversion, recurrence occurred later on with metastasis to the omentum and the small bowel, as well as intraperitoneal spread

Summary

Introduction

The plasmacytoid variant of urothelial carcinoma (PVUC) makes up a rare subtype of urothelial cancers. The plasmacytoid variant is characterized by tumor cells that resemble plasma cells and/or monocytes, as well as a variable number of single cells with the appearance of signet ring cells [3]. The prognosis of this variant is often poor due to the commonly advanced disease presentation upon diagnosis warranting aggressive treatments [1,4,5]

Case Series

Findings

Discussion

Conclusions