Introduction/Background*Perivascular epithelioid cells tumors (PEComas) are rare mesenchymal neoplasms composed by perivascular epithelioid cells that express both melanocytic and myogenic markers at the anatomopathological and immunohistochemical studies.

PEComas family is composed by angiomyolipomas, pulmonary clear cell “sugar” tumors, lymphangioleiomyomatosis, primary extrapulmonary sugar tumors and clear cell tumors of falciform ligament. In the 25% of the cases PEComas involve the female genital tract, the uterus is the most likely affected organ and the vagina the least.

This disease is mostly benign, however, there are cases of PEComas with local and distant metastasis at diagnosis. Thus, some risk scores have been developed to predict the benignity, malignancy or uncertain potential of each tumor.

Because of the rarity of such tumors, the diagnosis is usually histological, although recently common sonographic features have been defined.

Treatment is essentially surgical with the possibility of using chemotherapy for locally advanced or recurrent disease.

Result(s)*We report a case of a 73 years old woman referred to our hospital for a sense of vaginal weight; the gynecological examination revealed a 4 cm paravaginal mass. Ultrasound showed a hypoechogenic solid tumor with smooth contour, moderately vascularized (Color Score 3), in the right paravaginal space. The mass was adherent to the vaginal wall and hypomobile to the wall of the rectum and to the pelvic floor with no obvious infiltration of these structures.

The MRI study described a 4-cm-large mass with inhomogeneous vascularization at the level of the right posterolateral vaginal wall that imprinted the right elevator ani muscle without a clear plane of cleavage and, posteriorly, the anterior wall of the rectum with normal plane of cleavage.

The histological examination of the tumor revealed a PEComa. No other localizations were seen in CT scan either in pelvis or in upper abdomen; a total abdominal hysterectomy and bilateral salpingo-ophorectomy was performed to reach the mass located inside the right paravaginal-pararectal space on pubococcigeal muscle. The mass was then totally removed.

Conclusion*We report this case because of the exceptional localization of the PEComa and the peculiarity of the radiological study.