CT and clinical findings of peripheral primitive neuroectodermal tumour in children

作者：Huijuan Xiao, Fengchang Bao, Hongna Tan, Bo Wang, Wei Liu, Jianbo Gao, Xianzheng Gao · 发表于：British Journal of Radiology · 年份：2016 · DOI：10.1259/bjr.20140450 · 被引用次数：12 · 研究领域：Sarcoma Diagnosis and Treatment、Oral and Craniofacial Lesions、Oral and Maxillofacial Pathology

OBJECTIVE: To describe the clinical, CT and pathological findings of paediatric peripheral primitive neuroectodermal tumours (pPNETs) to enhance the recognition of these rare tumours. METHODS: The clinical, CT and pathological findings of 18 paediatric patients with pPNETs confirmed by biopsy or surgical pathology were retrospectively reviewed. RESULTS: The age of these 18 paediatric patients with pPNETs ranged from 4 months to 15 years, with a mean age of 7.7 years. The lesions of these 18 paediatric patients with pPNETs were located in the head and neck (n = 4), chest (n = 2), abdomen and pelvic cavity (n = 6), spine (n = 3), ilium (n = 2) and femur (n = 1). Immunohistochemical examination revealed Homer-Wright rosettes in seven lesions, and 94.4% of lesions showed consistent positive staining for CD99. On plain CT images, the majority of pPNETs showed lesions that were ill-defined (72.2%), irregularly shaped (83.3%), heterogeneous (66.7%) or hypodense masses (94.4%), and together with osteolytic bone destruction when the lesion originated in the bone. Calcifications were found in three lesions. After contrast administration, all soft-tissue masses were persistently enhanced heterogeneously with various cystic or necrotic regions, and 71.4% of them had linear enhancement. 94.4% of soft-tissue masses showed a moderate degree of enhancement. Seven cases had lymph node metastasis at diagnosis. CONCLUSION: Paediatric pPNET can involve any part of the body, and a large, ill-defi...