UTERINE PECOMA WITH CRITERIA FOR MALIGNANCY: CASE REPORT

Presentation: Woman, 44 years, submitted to a hysterectomywithadnexectomyin04/2018fortreatment of uterine myomatosis. Previously to surgery, she had a clinical condition of hypermenorrhea/menorrhagia. The anatomopathological report of the uterus showed intramural leiomyomas and a fusocellular/ epithelioid tumor amid collagenous stroma, measuring 24×22×7cm, affecting uterus and uterine appendages. The immunohistochemistry showed coexpression of muscular (desmin and actin) and melanocytic (HMB-45) markers, indicating the myelomelanocytic origin of the tumor and the diagnosis of perivascular epithelioid cell tumor (PEComa). The cellular proliferation index (Ki-67) was less than 1% and there werent found any sign of necrosis and atypia. After clinical evaluation and presence of expansive mass with the size of 13×3×9cm in pelvic TC, the patient was submitted to laparotomy in 05/2019. The tumor was unresectable, occupying all the left part of pelvis, invading bladder and sigmoid colon. The biopsy confirmed retroperitoneal PEComa with focus of supurative acute inflammation and absence of ominous morphologic signals. Discussion: The PEComas are rare mesenchymal tumors composed by perivascular epithelioid cells with immunoreactivity for melanocytic or/and smooth muscle markers, in this patients case both were positive. These tumors can mimetize others neoplasms as leiomyossarcomas and leiomyomas, that were important differential diagnosis in this patient. The literature reports about 100 cases of PEComas, and 1/3 had origin in the uterus. These tumors show variable prognosis, and, accord of the classification of gynecological PEComas, the patients neoplasm presented criteria for malignancy (size > 5cm and infiltrative growing pattern).The chosen treatment is surgical, as the adopted in this case, and the use of chemotherapy and radiotherapy in high-risk tumors, even with few randomized trials. Final comments: The uterine PEComa is a rare pathology and the prognosis is extremely variable. The presented case, as a high-risk tumor, advocates a careful state of vigilance and following-up on behalf of scarcity of information about that neoplasm and the variability of possible evolutions to the tumor.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
23 October 2019