Primitive Neuroectodermal Tumor of the Uterus: A Case Report

PNETs belong to the Ewing's sarcoma family. They are closely related to malignant, small round-cell tumors of soft tissue and bones. They strongly express the glycoprotein p30/32 (CD99) on the cell membrane, which is encoded by the MIC2 gene. A translocation at t(11;22)(q24;q12) has been identified. PNETs are mostly recognized by the histologic characteristics of neural differentiation and immunohistochemical expression of at least two different neural markers. Uterine PNETs have a poor prognosis and, to date, there is no standard treatment. Total abdominal hysterectomy and bilateral salpingo-oophorectomy plus adjuvant chemotherapy or radiotherapy are recommended.