Department 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.
Sci Rep. 2020 Sep 24;10(1):15656. doi: 10.1038/s41598-020-72680-6.
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.
外周原始神经外胚层肿瘤 (PNETs) 是一种非常罕见且具有侵袭性的恶性肿瘤。由于其在不同部位的发病率较低，目前尚无针对外周 PNETs 的标准治疗指南。治疗方法来源于尤文肉瘤家族，目前仍然是多模式的。我们的研究回顾性分析了 1998 年 2 月 1 日至 2018 年 2 月 1 日在北京协和医院就诊的 86 例 PNET 患者，并通过文献复习额外纳入了 75 例患者。分析了与生存相关的临床病理和治疗方案。手术、化疗、女性、肿瘤较小、无淋巴结转移、R0 手术切除、（长春新碱+多柔比星+环磷酰胺/异环磷酰胺+依托泊苷）方案、化疗周期数超过 10 个与单因素分析中总生存改善相关。手术、化疗周期数超过 10 个和肿瘤较小是总生存的独立预后因素。我们的数据表明，多模式治疗是外周 PNET 的主要治疗方法。
