Clinico-pathological features of gynecological myopericytoma: a challenging diagnosis in an exceptional location.

Abstract

Myopericytomas (MPC) are rare mesenchymal tumors, originating from the perivascular myoid cells. They predominantly occur in the skin and superficial soft tissues of the extremities, while visceral involvement is rare. Histological features and clinical course are usually benign. To the best of our knowledge, MPC is still an uncharacterized tumor entity of the female internal genital tract. We describe three MPC cases involving the female internal genital tract: (1) a uterine wall MPC arising in a 49-year-old woman with progressive pelvic/abdominal pain; (2) a cervix MPC of a 49-year-old woman who presented with metrorrhagia, and (3) a MPC presenting as a simple ovarian cyst in a 26-year-old woman with pain located in the left iliac fossa. All patients were surgically treated, and recurrence occurred in two cases. The histological and immunohistochemical findings, supporting the diagnosis of MPC, are presented; in particular, one case showed characteristics pointing towards an uncertain biological behavior/low-grade malignancy. A literature search was conducted to identify previous reports of gynecological MPC and for possible alternative diagnoses. Leiomyoma, epithelioid leiomyoma, angioleiomyoma, perivascular epithelioid cell tumor, solitary fibrous tumor, and low-grade endometrial stromal sarcoma should be considered in the differential diagnosis. Awareness of possible occurrence of this rare neoplasm in the female genital tract is important to reach a correct diagnosis in the spectrum of mesenchymal tumors. Considering the risk of recurrence, we recommend careful evaluation of surgical margins and complete surgical removal whenever possible.