Open AccessPeripheral primitive neuroectodermal tumor of the ovary with torsion
Author(s) -
Ben-Shian Huang,
Huann-Cheng Horng,
ChiungRu Lai,
Wen-Hsun Chang,
Wen-Hsiang Su,
MingShyen Yen,
Peng-Hui Wang
Publication year - 2013
Publication title -
gynecology and minimally invasive therapy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.441
H-Index - 12
eISSN - 2213-3089
pISSN - 2213-3070
DOI - 10.1016/j.gmit.2013.02.001
Subject(s) - medicine , vincristine , fertility preservation , ovary , cyclophosphamide , fertility , surgery , stage (stratigraphy) , chemotherapy , biology , paleontology , population , environmental health
Peripheral primitive neuroectodermal tumors (pPNETs) of the ovary are rare monophasic teratomas, and fewer than 100 cases have been reported in the literature. pPNETs mainly involve young women during their reproductive age, therefore, accurate diagnosis followed by multimodal treatment should be taken into consideration for fertility preservation. We report a patient with stage IA pPNET of the ovary presenting with acute abdominal pain secondary to torsion that was successfully managed by fertility-sparing surgery and six courses of combination chemotherapy with vincristine, Adriamycin, and cyclophosphamide. She has had a disease-free survival of >3 years. This brief review demonstrates the clinical course of pPNET and summarizes the literature to show that clinical stage at the time of diagnosis is the most important prognostic factor and that the vast majority of recurrences are observed within 10 years