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Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor
Yidi Liu1, Yan Yuan1, Fuquan Zhang1
1Department of Radiation Oncology, Peking Union Medical College Hospital. Chinese Academy of Medical Sciences & Peking Union Medical College, No.1 Shuaifuyuan Wangfujing, Dongcheng District, Beijing, 100730, People's Republic of China.
Abstract:
Peripheral primitive neuroectodermal tumors (PNETs) constitute very rare and aggressive malignancies. To date, there are no standard guidelines for management of peripheral PNETs due to the paucity of cases arising in various body sites. Therapeutic approach is derived from Ewing sarcoma family, which currently remains multimodal. Our study retrospectively analyzed 86 PNET patients from February 1, 1998 to February 1, 2018 at Peking Union Medical College Hospital with an additional 75 patients from review of literature. The clinicopathologic and treatment plans associated with survival was investigated. Surgery, chemotherapy, female sex, small tumor size, no lymph node metastasis, R0 surgical resection, (vincristine + doxorubicin + cyclophosphamide)/(isophosphamide + etoposide) regimen, and more than 10 cycles of chemotherapy were associated with improved overall survival in univariate analysis. Surgery, more than 10 cycles of chemotherapy, and small tumor size were independent prognostic factors for higher overall survival. Our data indicates that multimodal therapy is the mainstay therapeutic approach for peripheral PNET.
Insights
Peripheral primitive neuroectodermal tumors (PNETs) are rare, aggressive cancers lacking standard treatment guidelines. Multimodal therapy, including surgery and chemotherapy, is crucial for improving survival outcomes in PNET patients.
Area of Science:
- Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Peripheral primitive neuroectodermal tumors (PNETs) are rare and aggressive malignancies.
- Lack of established management guidelines due to limited case numbers.
- Treatment strategies often adapt from Ewing sarcoma family protocols, emphasizing multimodal approaches.
Purpose of the Study:
- To investigate clinicopathologic features and treatment plans associated with survival in peripheral PNET patients.
- To identify prognostic factors influencing overall survival in peripheral PNET.
- To evaluate the efficacy of multimodal therapy for peripheral PNET.
Main Methods:
- Retrospective analysis of 86 peripheral PNET patients from Peking Union Medical College Hospital (1998-2018).
- Inclusion of an additional 75 patients from literature review.
- Univariate and multivariate analyses to identify factors associated with improved overall survival.
Main Results:
- Improved overall survival was associated with surgery, chemotherapy, female sex, small tumor size, no lymph node metastasis, R0 resection, specific chemotherapy regimens (VDC/IE), and >10 cycles of chemotherapy in univariate analysis.
- Surgery, >10 cycles of chemotherapy, and small tumor size were identified as independent prognostic factors for higher overall survival.
- Multimodal therapy demonstrated a positive impact on patient outcomes.
Conclusions:
- Multimodal therapy, encompassing surgery and chemotherapy, is the primary treatment approach for peripheral PNET.
- Key prognostic factors for improved survival include surgical resection, extended chemotherapy cycles, and smaller tumor size.
- Further research into standardized treatment protocols for peripheral PNET is warranted.
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