Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature
Abstract Background 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 Eng...
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| Format: | Article |
| Language: | English |
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BMC
2019-06-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-019-1106-5 |
| _version_ | 1818970663984037888 |
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| author | Xiaopei Chao Yalan Bi Lei Li |
| author_facet | Xiaopei Chao Yalan Bi Lei Li |
| author_sort | Xiaopei Chao |
| collection | DOAJ |
| description | Abstract Background 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. Results Finally 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. Conclusions No standard therapy for ovarian primary PNET exists, and an individualized strategy is recommended. Young patients seem to have better prognoses. |
| first_indexed | 2024-12-20T14:40:04Z |
| format | Article |
| id | doaj.art-d2be03a632854476b1dd64238dcdf1ad |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-20T14:40:04Z |
| publishDate | 2019-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-d2be03a632854476b1dd64238dcdf1ad2022-12-21T19:37:20ZengBMCOrphanet Journal of Rare Diseases1750-11722019-06-011411910.1186/s13023-019-1106-5Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literatureXiaopei Chao0Yalan Bi1Lei Li2Department of Obstetrics and Gynecology, Peking Union Medical College HospitalDepartment of Pathology, Peking Union Medical College HospitalDepartment of Obstetrics and Gynecology, Peking Union Medical College HospitalAbstract Background 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. Results Finally 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. Conclusions No standard therapy for ovarian primary PNET exists, and an individualized strategy is recommended. Young patients seem to have better prognoses.http://link.springer.com/article/10.1186/s13023-019-1106-5Ovary tumorPrimary neuroectodermal tumorChemotherapyRadiotherapyTarget therapyPrognosis |
| spellingShingle | Xiaopei Chao Yalan Bi Lei Li Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature Orphanet Journal of Rare Diseases Ovary tumor Primary neuroectodermal tumor Chemotherapy Radiotherapy Target therapy Prognosis |
| title | Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature |
| title_full | Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature |
| title_fullStr | Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature |
| title_full_unstemmed | Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature |
| title_short | Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature |
| title_sort | ovarian primary primitive neuroectodermal tumor a review of cases at pumch and in the published literature |
| topic | Ovary tumor Primary neuroectodermal tumor Chemotherapy Radiotherapy Target therapy Prognosis |
| url | http://link.springer.com/article/10.1186/s13023-019-1106-5 |
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