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

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.

Object Details

• Alternative title: Entidade ginecológica rara: PEComa cervical maligno (tumor perivascular de diferenciação de células epitelióides) - desafios no diagnóstico, tratamento e vigilância
• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch

References and Relationships

• Bibliographic citation: Rare gynecological entity: malignant cervical PEComa (perivascular epithelioid cell differentiation tumour) - challenges in diagnosis, treatment and surveillance ; volume:20 ; number:CP ; year:2024 ; Brazilian journal of oncology ; 20, Heft CP (2024)

Contributors, Places and Time

• Contributor: Anjos, Amanda da Silva ; Kwiatkowski, Filipe Vieira ; Kwiatkowski, Rafael Vieira ; Bonow, Danielle Rediess ; Vasques, Luana Ferreira ; Teixeira, Sabine Triguero

Further information

• DOI: 10.5935/2526-8732.20230459
• URN: urn:nbn:de:101:1-2501301155384.832238451677
• Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 15.08.2025, 7:24 AM CEST