Case Report


Uterine perivascular epithelioid cell neoplasm (PEComa) in a young woman: Case report, therapeutic considerations, and literature review

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1 MD, Hospital das Clínicas, Federal University of Pernambuco, Recife, PE, Brazil

2 Adjunct Professor of Gynecology, Department of Gynecology-Obstetrics, Federal University of Pernambuco, Recife, PE, Brazil

3 PhD Candidate, LIKA – Laboratório de Imunopatologia Keizo Assami, Federal University of Pernambuco, Recife, PE, Brazil

4 Undergraduate student, Medical Sciences Center, Federal University of Pernambuco, Recife, PE, Brazil

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Iolanda Matias Gomes

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Article ID: 101259Z01IG2021

doi: 10.5348/101259Z01IG2021CR

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How to cite this article

Gomes IM, Cirino LMD, de Oliveira MS, Ramalho EFR, de Queiroz Júnior JRA, Melo IO, Ferreira CCOB, de Moura SRS. Uterine perivascular epithelioid cell neoplasm (PEComa) in a young woman: Case report, therapeutic considerations, and literature review. Int J Case Rep Images 2021;12:101259Z01IG2021.

ABSTRACT


Introduction: Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor in woman’s genital tract, mainly uterus. Its potential for malignancy, also the therapy, is still not clear. It frequently occurs in fourth decade of life and treatment is often surgical. When it occurs in young women desirous of fertility, it brings dilemmas as to which is the best treatment to guarantee the good chance of survival.

Case Report: A 24-year-old woman complaining of severe genital bleeding for four months and an ultrasound examination showing a 2.6 cm pathological growth in the uterine cavity, of endometrial polyp. She had a history of cesarean delivery. Colposcopy and Pap smear were normal. The lesion was completely resected from its base and the postoperative period had no complications. The result of the histopathological study showed a macroscopic lesion of 12 g, 3.0×0.6×0.3 cm, whose microscopy corresponded to a PEComa. As it was a rare tumor with risk of metastases, it was performed the hysterectomy with salpingectomy. The histopathology result showed a myometrium which had a residual lesion extended up to 0.3 cm short of the uterine serosa.

Conclusion: The authors propose is to perform hysterectomy with salpingectomy for primary uterus PEComas without estrogen and progesterone receptors. The patient has been in remission for 15 months and has maintained her ovarian activity.

Keywords: Female, Genital neoplasms, Hysterectomy, Immunohistochemistry, Rare tumor, Uterus

SUPPORTING INFORMATION


Author Contributions

Iolanda Matias Gomes - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Final approval of the version to be published

Lorena Magale Dantas Cirino - Substantial contributions to conception and design, Drafting the article, Final approval of the version to be published

Márcia Silva de Oliveira - Acquisition of data, Drafting the article, Final approval of the version to be published

Edneide Florivalda Ramos Ramalho - Revising it critically for important intellectual content, Final approval of the version to be published

José Reginaldo Alves de Queiroz Júnior - Acquisition of data, Drafting the article, Final approval of the version to be published

Isadora Oliveira Melo - Acquisition of data, Drafting the article, Final approval of the version to be published

Caio César Odijas Barbosa Ferreira - Acquisition of data, Drafting the article, Final approval of the version to be published

Sérgio Ricardo Soares de Moura - Acquisition of data, Drafting the article, Final approval of the version to be published

Guaranter of Submission

The corresponding author is the guarantor of submission.

Source of Support

None

Consent Statement

Written informed consent was obtained from the patient for publication of this article.

Data Availability

All relevant data are within the paper and its Supporting Information files.

Conflict of Interest

Authors declare no conflict of interest.

Copyright

© 2021 Iolanda Matias Gomes et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.