García GOF, Nicolae A, Cuevas BM, García A, Nogales FF

Language: Spanish
References: 28
Page: 327-331
PDF size: 245.79 Kb.

ABSTRACT

Background: Perivascular epithelioid cells tumors (PEComas) conform a rare group of tumors that have been described preferently in the uterus.
Objective: To report a case of malignant uterine perivascular epithelioid cell tumor in a 40-years-old woman.
Material and methods: Pelvic ultrasonography revealed a polypoid mass in uterus. Pipelle biopsy showed a secretory endometrium with decidual change containing tumor with atypical epithelioid cells and abundant extracellular hyaline material.
Results: The differential diagnoses considered according with the age of the patient and histologic findings were lesions of the intermediate trophoblast (epithelioid trophoblastic tumor, placental site trophoblastic tumor and exaggerated placental site), choriocarcinoma and leiomiosarcoma. The immunohistochemical study showed positivity for smooth muscle and HMB45, melan-A and smooth muscle actin with a proliferative index Ki67 of 50%.
Discussion: Biopsy results were determinant of further clinical workup that prompted the diagnosis of a tuberous sclerosis complex.
Conclussions: The complementary studies have been clearance of these tumors. We suggest considered an isolated entity, by association to tuberous sclerosis complex, like this patient.

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