Division of Surgical Oncology, The Hiram C. Polk Department of Surgery, University of Louisville School of Medicine, Louisville, Kentucky.
Department of Surgery, Division of Surgical Oncology, Cooper University Hospital, Camden, New Jersey.
J Surg Oncol. 2020 Nov;122(6):1145-1151. doi: 10.1002/jso.26139. Epub 2020 Jul 30.
Primitive neuroectodermal tumors (PNETs) comprise less than 1% of all sarcomas. The rarity of this disease has resulted in a paucity of information about disease process and management. This study sought to evaluate the incidence, treatment patterns, and outcomes among patients with PNET.
The National Cancer Database was queried for diagnoses of PNET between 2004 and 2014. Patients were dichotomized based on tumor type (central [cPNET] vs peripheral [pPNET]). Demographic, tumor, treatment, and outcome variables were analyzed for the entire patient cohort and by type of PNET.
White (86.4%) males (56.6%) represented the majority of patients. The incidence of PNET remained stable over the study period (r = 0.0821). A total of 70.7% underwent surgical resection of the primary site, 50.3% received radiation, and 74.7% received systemic chemotherapy. Compared to those with pPNET, patients with cPNET more often received radiation treatment (P < .001), primary tumor resection (P < .001), and experienced increased 90-day mortality (P < .014).
cPNET and pPNET are rare and aggressive malignancies that tend to arise in White males. Multimodal treatment including surgery, chemotherapy, and radiation is conventional. Patients with cPNET more often receive radiation and primary tumor resection with increased 90-day mortality.
原始神经外胚层肿瘤（PNET）占所有肉瘤的比例不到 1%。这种疾病的罕见性导致人们对其疾病过程和管理知之甚少。本研究旨在评估 PNET 患者的发病率、治疗模式和结局。
2004 年至 2014 年期间，国家癌症数据库对 PNET 的诊断进行了查询。根据肿瘤类型（中枢[cPNET]与外周[pPNET]）将患者分为两类。对整个患者队列和两种 PNET 类型的患者进行了人口统计学、肿瘤、治疗和结局变量的分析。
白人（86.4%）男性（56.6%）占大多数患者。研究期间 PNET 的发病率保持稳定（r=0.0821）。共有 70.7%的患者接受了原发部位的手术切除，50.3%的患者接受了放疗，74.7%的患者接受了全身化疗。与 pPNET 患者相比，cPNET 患者更常接受放疗（P<.001）、原发肿瘤切除术（P<.001），并经历了更高的 90 天死亡率（P<.014）。
cPNET 和 pPNET 是罕见且侵袭性的恶性肿瘤，多发生于白人男性。包括手术、化疗和放疗在内的多模式治疗是常规治疗。cPNET 患者更常接受放疗和原发肿瘤切除术，90 天死亡率更高。
