Abstract
BackgroundThe pathological characteristics, treatment strategies and prognosis of ovarian primary primitive neuroectodermal tumor (PNET) were unclear due to the rarity of PNET. All cases treated at Peking Union Medical College Hospital (PUMCH) between 1975 and 2016 and published in the English literature between 1980 to 2017 were reviewed.ResultsFinally four cases from PUMCH and 15 cases in the literature were included. The median age was 25 years (range 13–79), and the median diameter of the tumors was 13.4 cm (range 5.0–30.0). The most common initial symptoms were abdominal pain, bloating and a pelvic mass. Diagnosis primarily depended on immunohistochemical and fluorescence in situ hybridization data. Treatment consisted of surgery, various chemotherapy regimens and/or radiotherapy. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 15 and 52%, respectively. For patients with OS and PFS > 12 months, the median ages were 21 years (range 13–35) and 17 years (range 13–35), respectively, while for patients with OS < 12 months and PFS < 12 months, the median ages were 48 years (range 14–79) and 25 years (range 18–79), respectively.ConclusionsNo standard therapy for ovarian primary PNET exists, and an individualized strategy is recommended. Young patients seem to have better prognoses.
Highlights
Primitive neuroectodermal tumor (PNET) was first proposed by Hart and Earle [1] in 1973
According to World Health Organization (WHO) classification, ovarian primary primitive neuroectodermal tumor (PNET) can be divided into either tumors that resemble their central nervous system counterparts or peripheral PNET (pPNET)/Ewing’s sarcomas
We report four cases of ovarian primary PNET treated at Peking Union Medical College Hospital (PUMCH), Beijing, China, between 1975 and 2016 and 15 cases reported in the English literature between 1980 and 2017
Summary
Primitive neuroectodermal tumor (PNET) was first proposed by Hart and Earle [1] in 1973. PNET is classified as central PNET and peripheral PNET (pPNET), and the most common sites of the latter are the paravertebral region and the chest wall. Classification of pPNET depends primarily on the differentiation of its neural components. According to World Health Organization (WHO) classification, ovarian primary PNET can be divided into either tumors that resemble their central nervous system counterparts or pPNET/Ewing’s sarcomas. The pathological characteristics, treatment strategies and prognosis of ovarian primary primitive neuroectodermal tumor (PNET) were unclear due to the rarity of PNET. All cases treated at Peking Union Medical College Hospital (PUMCH) between 1975 and 2016 and published in the English literature between 1980 to 2017 were reviewed
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