Uterine Primitive Neuroectodermal Tumor

Uterine primitive neuroectodermal tumors (PNETs) are rare entities, with only around 70 cases published in the literature. Most of them are diagnosed in advanced stages with rapid progression and poor prognosis. Herein, we present a case of a 71-year-old patient with postmenopausal metrorrhagia and an ultrasound finding of endometrial thickening. The pathological diagnosis after an endometrial biopsy showed PNET. In the study of extension, possible distant dissemination with infiltration of the sigmoid and liver was observed. Chemotherapy treatment was proposed, but not begun due to the rapid progression of the disease. Four months after the initial diagnosis, the patient died of multiple organ failure. While there is no optimal chemotherapy treatment regimen for PNET, some studies have reported encouraging results. It is necessary to publish more studies emphasizing the follow-up and survival of the disease to establish which may be the best treatment option and thus improve the current poor prognosis.