Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects

Abstract Plasmacytoid (PUC) variant is a rare and aggressive form of urothelial cancer representing 1 to 3% of the bladder cancer. The main differential diagnosis is the bladder involvement by lymphoma-plasmocytoma or metastasis from lobular breast cancer or diffuse gastric cancer. Immunexpression o...

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Main Author: Katia Ramos Moreira Leite
Format: Article
Language:English
Published: BMC 2020-01-01
Series:Surgical and Experimental Pathology
Subjects:
Online Access:https://doi.org/10.1186/s42047-020-0056-5
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author Katia Ramos Moreira Leite
author_facet Katia Ramos Moreira Leite
author_sort Katia Ramos Moreira Leite
collection DOAJ
description Abstract Plasmacytoid (PUC) variant is a rare and aggressive form of urothelial cancer representing 1 to 3% of the bladder cancer. The main differential diagnosis is the bladder involvement by lymphoma-plasmocytoma or metastasis from lobular breast cancer or diffuse gastric cancer. Immunexpression of cytokeratin 7 and GATA3 is the rule, but CD138 may be positive in high percentage of cases. CDH1 somatic mutation or, more rarely, methylation of the gene promoter is the main genetic characteristic of PUC, but germinative mutation is always negative. The recognition of this special histology is very important for the correct management of the patients because of the high rate of positive surgical margins and atypical disease progression. PUC is responsive to cisplatin-based chemotherapy but recurrence is the rule. Peritoneal dissemination is frequent and cancer specific mortality is as high as 56% in a range of 19 to 23 months.
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spelling doaj.art-10fb99a1dba14fbb99edcba54d9c776e2022-12-21T21:33:21ZengBMCSurgical and Experimental Pathology2520-84542020-01-01311510.1186/s42047-020-0056-5Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspectsKatia Ramos Moreira Leite0Laboratory of Medical Investigation – LIM55, Urology Department, University of Sao Paulo Medical SchoolAbstract Plasmacytoid (PUC) variant is a rare and aggressive form of urothelial cancer representing 1 to 3% of the bladder cancer. The main differential diagnosis is the bladder involvement by lymphoma-plasmocytoma or metastasis from lobular breast cancer or diffuse gastric cancer. Immunexpression of cytokeratin 7 and GATA3 is the rule, but CD138 may be positive in high percentage of cases. CDH1 somatic mutation or, more rarely, methylation of the gene promoter is the main genetic characteristic of PUC, but germinative mutation is always negative. The recognition of this special histology is very important for the correct management of the patients because of the high rate of positive surgical margins and atypical disease progression. PUC is responsive to cisplatin-based chemotherapy but recurrence is the rule. Peritoneal dissemination is frequent and cancer specific mortality is as high as 56% in a range of 19 to 23 months.https://doi.org/10.1186/s42047-020-0056-5Urinary bladderPlasmacytoidUrothelial carcinomaBladderVariantImmunohistochemistry
spellingShingle Katia Ramos Moreira Leite
Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects
Surgical and Experimental Pathology
Urinary bladder
Plasmacytoid
Urothelial carcinoma
Bladder
Variant
Immunohistochemistry
title Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects
title_full Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects
title_fullStr Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects
title_full_unstemmed Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects
title_short Plasmocytoid urothelial carcinoma - clinical, histological, immunohistochemical and molecular aspects
title_sort plasmocytoid urothelial carcinoma clinical histological immunohistochemical and molecular aspects
topic Urinary bladder
Plasmacytoid
Urothelial carcinoma
Bladder
Variant
Immunohistochemistry
url https://doi.org/10.1186/s42047-020-0056-5
work_keys_str_mv AT katiaramosmoreiraleite plasmocytoidurothelialcarcinomaclinicalhistologicalimmunohistochemicalandmolecularaspects