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Ginecología y Obstetricia de México

Federación Mexicana de Ginecología y Obstetricia, A.C.
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2021, Number 05

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Ginecol Obstet Mex 2021; 89 (05)

Uterine PEComa: Perivascular epithelioid cell neoplasm

Garza-Hernández C, Treviño-Salinas EM, Barboza-Quintana O, Caballero-Malacara V, Rodríguez-Contreras MR
Full text How to cite this article

Language: Spanish
References: 6
Page: 405-408
PDF size: 199.15 Kb.


Key words:

PEComa, Perivascular epithelioid cell neoplasms, Uterine body, Hysterectomy, Uterus, Menstrual cycle.

ABSTRACT

Background: PEComa is a perivascular epithelioid cell tumor of mesenchymal origin that expresses melanocytic and myogenic markers. These tumors are a rare group of neoplasms. There are about 100 reported cases of which 30% are gynecologic and the uterine body is the most frequent site. Due to the low frequency of occurrence, there is no established medical management and treatment.
Clinical case: A 47-year-old female patient attended the Hospital Universitario Dr. José Eleuterio González due to dysmenorrhea and alteration of the menstrual cycle characterized by an increase in quantity, of one year of evolution. The pelvic ultrasound showed uterine myomatosis; abdominal hysterectomy was decided. The anatomopathological report was perivascular epitheloid cell tumor (PEComa) with which the diagnosis of PEComa of malignant potential was established.
Conclusion: PEComa is a rare neoplasm since only a few gynecologic cases have been reported. It is important to know its macro and microscopic characteristics to establish an adequate diagnosis and treatment.


REFERENCES

  1. Bonetti F, Martignoni G, Colato C, et al. Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol. 2001;14:563-568. https://doi.org/10.1038/ modpathol.3880351.

  2. Musella A, De Felice, Kyriacou AK, Barletta F. Perivascular epithelioid cell neoplasm (PEComa) of the uterus: a systematic review. Int J of Surg. 2015; 19: 1-5. doi.org/10.1016/j. ijsu.2015.05.002.

  3. Agrawal P, Anagani M. Uterine PEComa - A Group of Rare Mesenchymal Tumors. J Minim Invasive Gynecol. 2020; 27 (4): 803-4. https://doi.org/10.1016/j.jmig.2019.08.020.

  4. Rothenberger, R. et al. An unusual case of uterine PEComa presenting with disseminated intravascular coagulation. Gynecol Oncol Rep. 2018; 29: 76-78. https://doi. org/10.1016/j.gore.2019.06.007.

  5. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010; 41 (1): 1-15. https://doi.org/10.1016/j.humpath.2009.05.011.

  6. Shan W, Shi Y, Zhu Q, et al. Five cases of uterine perivascular epithelioid cell tumors (PEComas) and review of literature. Arch. Gynecol. Obstet. 2019; 299 (1): 185-90. https:// doi.org/10.1007/s00404-018-4920-4.




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Ginecol Obstet Mex. 2021;89