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CC BY 4.0 · Brazilian Journal of Oncology 2024; 20: e-20230459
DOI: 10.5935/2526-8732.20230459
Case Report
Surgical Oncology

Rare gynecological entity: malignant cervical PEComa (perivascular epithelioid cell differentiation tumour) - challenges in diagnosis, treatment and surveillance

Entidade ginecológica rara: PEComa cervical maligno (tumor perivascular de diferenciação de células epitelióides) - desafios no diagnóstico, tratamento e vigilância
Amanda da Silva Anjos 
1   Faculdades Pequeno Príncipe, Medicine, Curitiba, Paraná, Brazil
,
Filipe Vieira Kwiatkowski 
2   Santa Casa de Misericórdia de Pelotas, Surgical Oncology, Pelotas, Rio Grande do Sul, Brazil
,
Rafael Vieira Kwiatkowski 
2   Santa Casa de Misericórdia de Pelotas, Surgical Oncology, Pelotas, Rio Grande do Sul, Brazil
,
Danielle Rediess Bonow 
3   Universidade Federal de Rio Grande, Medicine, Rio Grande, Rio Grande do Sul, Brazil
,
Luana Ferreira Vasques 
4   Universidade Católica de Pelotas, Medicine, Pelotas, Rio Grande do Sul, Brazil
,
Sabine Triguero Teixeira 
5   Universidade Nove de Julho, Medicine, São Paulo, São Paulo, Brazil
› Author Affiliationsnone to declare
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ABSTRACT

Perivascular epithelioid cell tumors constitute a family of mesenchymal tumors characterized by the concomitant expression of melanocytic and muscular markers. Gynecological perivascular epithelioid cell tumors are rare, encompassing about 25% of cases. Studies demonstrate an even rarer occurrence in the uterine cervix. In this article, we report a case of malignant perivascular epithelioid cell tumors of the uterine cervix in a young patient, managed with total hysterectomy with unilateral salpingo- oophorectomy (due to suspected neoplasic involvement of the right ovary) and bilateral pelvic lymphadenectomy. Due to limited data, diagnosing these tumors is challenging. Given the uncertain biological behavior of this neoplasm, they should be considered potentially malignant and require long-term follow-up, despite the potential for late local recurrence and distant metastases. Surgical treatment involving complete resection of the lesion with clear margins remains the recommended option for this type of tumor until more consistent evidence can support adjuvant treatments.

RESUMO

Os tumores perivasculares de células epitelióides constituem uma família de tumores mesenquimais caracterizados pela expressão concomitante de marcadores melanocíticos e musculares. Tumores perivasculares de células epitelióides ginecológicos são raros, abrangendo cerca de 25% dos casos. Estudos demonstram uma ocorrência ainda mais rara no colo uterino. Neste artigo, relatamos um caso de tumor perivascular de células epitelióides maligno do colo uterino em paciente jovem, tratado com histerectomia total com salpingo-ooforectomia unilateral (por suspeita de envolvimento neoplásico do ovário direito) e linfadenectomia pélvica bilateral. Devido aos dados limitados, diagnosticar esses tumores é um desafio. Dado o comportamento biológico incerto desta neoplasia, devem ser consideradas potencialmente malignas e requerem seguimento a longo prazo, apesar do potencial de recorrência local tardia e metástases à distância. O tratamento cirúrgico envolvendo ressecção completa da lesão com margens claras continua sendo a opção recomendada para esse tipo de tumor até que evidências mais consistentes possam apoiar tratamentos adjuvantes.

Keywords:

Uterine cervical neoplasms - Perivascular epithelioid cell neoplasms - Hysterectomy

Descritores:

Neoplasias cervicais uterinas - Neoplasias perivasculares de células epitelióides - Histerectomia


Publication History

Received: 04 December 2023

Accepted: 05 February 2024

Article published online:
02 May 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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Bibliographical Record
Amanda da Silva Anjos, Filipe Vieira Kwiatkowski, Rafael Vieira Kwiatkowski, Danielle Rediess Bonow, Luana Ferreira Vasques, Sabine Triguero Teixeira. Rare gynecological entity: malignant cervical PEComa (perivascular epithelioid cell differentiation tumour) - challenges in diagnosis, treatment and surveillance. Brazilian Journal of Oncology 2024; 20: e-20230459.
DOI: 10.5935/2526-8732.20230459
 

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