Primitive neuroectodermal tumor of the uterus. A case report

Abstract

Background: Primitive neuroectodermal tumors (PNETs) of the uterus are very rare. Even preoperative curettage specimen morphology does not permit establishing a proper histogenetic diagnosis.

Case: A 16-year-old woman was admitted to the hospital because of metrorrhagia of three months' duration. The curettage specimen was interpreted as poorly differentiated sarcoma. The patient was operated on, and a total abdominal hysterectomy with bilateral salpingo-oophorectomy with omentectomy was performed. Histologically the tumor was composed of uniform, rounded, oval and sometimes spindle shaped cells with a narrow rim of eosinophilic cytoplasm. The cells were positive for neurogenic marker protein gene product, neuron-specific enolase and Ewing's sarcoma-related HBA-71. All cells were uniformly negative for Ber EP4 monoclonal antibody recognizing an epithelium-specific surface antigen. A diagnosis of PNET was rendered. The patient received combined therapy, external radiation to the pelvis and chemotherapy. Four years later she was alive, without signs of recurrent tumor.

Conclusion: In spite of the generally recognized aggressive behavior of PNET, it can have a long disease-free survival rate when appropriately treated.