Abstract

Primitive neuroectodermal tumor (PNET) arise from Kulchitsky cells and are rarely seen in the female genital tract. Differential diagnosis of PNET can be made based on immunohistochemical profiles and genetic analyses. Genital tract pNETs are very aggressive pathologies with different clinical and molecular manifestations and there are no standard guidelines for treatment. We aimed to present two cases of uterine PNETs with different symptomatology and clinical findings.

Highlights

  • Primitive neuroectodermal tumors (PNET) of the female genital tract are rarely observed

  • Some of the uterine PNETs are associated with other tumor components such as endometrioid adenocarcinoma, carcinosarcoma, unclassified sarcoma, rhabdomyosarcoma, and adenosarcoma(3)

  • Our cases were not associated with other tumor components but 15 cases with other associated uterine pathologies were reported in the last review article(2)

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Summary

Introduction

Primitive neuroectodermal tumors (PNET) of the female genital tract are rarely observed. The term PNET was first used by Hart and Earle in 1973 to introduce a group of tumors derived from fetal neuroectodermal cells(1). Ewing’s sarcoma and primitive neuroectodermal tumor (PNET) stand for a single group of bone and soft tissue tumours in which PNET with evidence of neural differentiation lies at one end of the spectrum, and undifferentiated Ewing’s sarcoma lies at the other. Both have similar phenotypes and share an identical chromosomal translocation. This study aimed to describe and discuss this rare pathology of the female genital tract

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