Dunne Richard F, Sahasrabudhe Deepak M, Messing Edward M, Jean-Gilles Jerome, Fung Chunkit
James P. Wilmot Cancer Center, Division of Hematology/Oncology, University of Rochester Medical Center , Rochester, NY, USA.
Department of Urology, University of Rochester Medical Center , Rochester, NY, USA.
Rare Tumors. 2014 Mar 18;6(1):5268. doi: 10.4081/rt.2014.5268. eCollection 2014 Jan 23.
Primitive neuroectodermal tumor (PNET) is a pathologic diagnosis that encompasses several different tumor types, including central nervous system tumors and Ewing's sarcomas. Teratoma, a common element of germ cell tumor (GCT), has the ability to transform to malignant PNET in a small number of patients. Making a definitive diagnosis of PNET is difficult given its deviation from elements of GCT and its non-specific pathologic findings. Establishing the diagnosis is crucial as PNETs respond poorly to standard platinum-based chemotherapy used for treatment of GCT. Primary treatment for PNET is surgical, though this is often not feasible in many patients due to extensive disease at diagnosis. As an alternative, chemotherapy regimens traditionally used for Ewing's sarcoma, such as vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide, have shown limited efficacy in the neoadjuvant, adjuvant, and palliative settings. Future research should delineate the genetic underpinnings of PNET and develop therapeutic options accordingly.
原始神经外胚层肿瘤（PNET）是一种病理诊断，涵盖几种不同的肿瘤类型，包括中枢神经系统肿瘤和尤因肉瘤。畸胎瘤是生殖细胞肿瘤（GCT）的常见组成部分，在少数患者中有转化为恶性PNET的能力。鉴于PNET与GCT成分不同且病理表现不具特异性，因此很难做出明确诊断。由于PNET对用于治疗GCT的标准铂类化疗反应不佳，所以明确诊断至关重要。PNET的主要治疗方法是手术，但由于诊断时疾病广泛，这在许多患者中往往不可行。作为替代方案，传统上用于尤因肉瘤的化疗方案，如长春新碱、阿霉素和环磷酰胺与异环磷酰胺和依托泊苷交替使用，在新辅助、辅助和姑息治疗中疗效有限。未来的研究应阐明PNET的遗传基础，并据此开发治疗方案。
