ReviewOrphanet journal of rare diseases2019
Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature.
Review in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed.
- Article
- Atypical Pelvic Tumors in Children.Cancers · 2025Article
- Bilateral primary ovarian Ewing sarcoma recurring as left submandibular lymphadenopathy diagnosed on cytology.Autopsy & case reports · 2024Article
- A Case of Primary Ovarian Primitive Neuroectodermal Tumor.Medical journal of the Islamic Republic of Iran · 2024Article
- Targeted treatment of refractory primitive neuroectodermal tumor arising from an immature teratoma with crizotinib leading to a sustained response.Clinical case reports · 2023Article
- Giant primitive neuroectodermal pelvic tumour: a case report and literature review.The Journal of international medical research · 2020Review
- Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade's Experience From a Single Centre in India.Clinical pathology (Thousand Oaks, Ventura County, Calif.)Article
- Primitive neuroectodermal tumors of the ovary: a multidecade review of the scientific literature.Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologieArticle
Corrections and comments
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Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
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.
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