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Case Report
Uterine perivascular epithelioid cell neoplasm (PEComa) in a young woman: Case report, therapeutic considerations, and literature review
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
Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife - PE, 50670-901,
Brazil
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Article ID: 101259Z01IG2021
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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 - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Lorena Magale Dantas Cirino - Conception of the work, Design of the work, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Márcia Silva de Oliveira - Acquisition of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Edneide Florivalda Ramos Ramalho - Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
José Reginaldo Alves de Queiroz Júnior - Acquisition of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Isadora Oliveira Melo - Acquisition of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Caio César Odijas Barbosa Ferreira - Acquisition of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Sérgio Ricardo Soares de Moura - Acquisition of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors 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.
