Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor.
Yidi LiuYan YuanFuquan ZhangKe HuJie QiuXiaorong HouJunfang YanXin LianShuai SunZhikai LiuJie ShenPublished in: Scientific reports (2020)
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.
Keyphrases
- prognostic factors
- end stage renal disease
- lymph node metastasis
- minimally invasive
- chemotherapy induced
- chronic kidney disease
- ejection fraction
- healthcare
- squamous cell carcinoma
- low dose
- pain management
- mesenchymal stem cells
- drug delivery
- emergency department
- machine learning
- clinical practice
- chronic pain
- high speed
- deep learning