Department of Radiology, All India Institute of Medical Sciences, New Delhi 110029, India.
Singapore Med J. 2013 Aug;54(8):463-2. doi: 10.11622/smedj.2013155.
Primitive neuroectodermal tumours (PNETs) arise from pluripotent neural crest cells and are classified as either central or peripheral. Peripheral PNETs (pPNETs) arise outside the central nervous system and sympathetic chain. These rare neoplasms comprise only 1% of all sarcomas and have highly aggressive biological behaviour and dismal prognosis. Adolescents and young adults are typically affected. Only isolated case reports on pPNETs appearing in both typical and atypical sites can be found in the literature. Timely diagnosis of pPNETs is a challenge to clinicians and radiologists due to the disease's insidious onset and variable locations, coupled with the limited studies that focus on the imaging features of pPNETs. Hence, this article serves to review the imaging features of this rare tumour.
原始神经外胚层肿瘤(PNETs)起源于多能神经嵴细胞,分为中枢性和外周性。外周性 PNET(pPNET)起源于中枢神经系统和交感神经链之外。这些罕见的肿瘤仅占所有肉瘤的 1%,具有高度侵袭性的生物学行为和预后不良。青少年和年轻成年人通常受到影响。文献中仅可发现一些关于出现在典型和非典型部位的 pPNET 的孤立病例报告。由于疾病的隐匿性发病和多变的部位,再加上针对 pPNET 影像学特征的有限研究,pPNET 的及时诊断对临床医生和放射科医生来说是一个挑战。因此,本文旨在回顾这种罕见肿瘤的影像学特征。