Vulvar "proximal-type" Epithelioid Sarcoma: Report of a Case and Review of the Literature

Overview

Journal Diagn Pathol

Publisher Biomed Central

Specialty Pathology

Date 2013 Jul 27

PMID 23886403

Citations 8

Authors

Lodovico Patrizi

Giacomo Corrado

Maria Saltari

Letizia Perracchio

Chiara Scelzo

Emilio Piccione

Enrico Vizza

Affiliations

Soon will be listed here.

Abstract

Background: The "proximal-type" epithelioid sarcoma is a very rare kind of mesenchimal tumor characterized by the difficulty in histological diagnosis and the very aggressive biological behavior.

Case: We report of a case of a 63 years old woman with a vulvar "proximal-type" epithelioid sarcoma that underwent a radical surgical staging followed by an adjuvant radiotherapy. She is on follow-up care for 14 months and there is no clinical evidence of disease.

Conclusion: Even if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of particularly aggressive biologic behaviour. Its diagnosis attracts controversies and criticism related to the surgical approach and the choice of an adjuvant therapy.

Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1508554852942125.

Citing Articles

Proximal-type epithelioid sarcoma of the perineum: A case report and literature review.

Xia S, Wu W, Ma L Front Oncol. 2023; 13:1057466.

PMID: 36950547 PMC: 10025383. DOI: 10.3389/fonc.2023.1057466.

Clinicopathological Characteristics, Treatment, and Survival in Patients Diagnosed With Proximal-Type Epithelioid Sarcoma: A Case Report and Systematic Review.

Zegarra Buitron E, Vidal Panduro D, Morales Luna D Cureus. 2023; 14(12):e32962.

PMID: 36712724 PMC: 9875881. DOI: 10.7759/cureus.32962.

A proximal type epithelioid sarcoma of the vulva with multiple distant metastases: A case report and review of the literature.

Chung H, Jang T, Kwon S, Ha J, Shin S Gynecol Oncol Rep. 2021; 37:100835.

PMID: 34377757 PMC: 8327478. DOI: 10.1016/j.gore.2021.100835.

SMARCB1/INI1-deficient tumors of adulthood.

Parker N, Al-Obaidi A, Deutsch J F1000Res. 2021; 9:662.

PMID: 33796273 PMC: 7968524. DOI: 10.12688/f1000research.24808.2.

An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures.

Jeon S, Lee J, Jung H, Kim B, Ryu S, Park S Obstet Gynecol Sci. 2020; 63(5):670-674.

PMID: 32727169 PMC: 7494770. DOI: 10.5468/ogs.20076.

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