Xiao Huijuan, Bao Fengchang, Tan Hongna, Wang Bo, Liu Wei, Gao Jianbo, Gao Xianzheng
1 Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Henan, China.
2 Department of Hematology, Children's Hospital of Zhengzhou City, Zhengzhou, Henan, China.
Br J Radiol. 2016;89(1060):20140450. doi: 10.1259/bjr.20140450. Epub 2016 Feb 5.
To describe the clinical, CT and pathological findings of paediatric peripheral primitive neuroectodermal tumours (pPNETs) to enhance the recognition of these rare tumours.
The clinical, CT and pathological findings of 18 paediatric patients with pPNETs confirmed by biopsy or surgical pathology were retrospectively reviewed.
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.
Paediatric pPNET can involve any part of the body, and a large, ill-defined, aggressive soft-tissue mass and moderate heterogeneous enhancement with varying cystic regions and linear enhancement, with or without osteolytic bone destruction, on CT images could suggest the diagnosis.
Primitive neuroectodermal tumours constitute a rare type of malignant neuroectodermal tumours that have chromosomal translocations identical to Ewing's sarcoma, and reports about radiological characteristics of this disease in children are insufficient. This study has described the clinical features and CT and pathological findings in 18 paediatric patients diagnosed with pPNETs in different locations, as a way to enhance the recognition of these tumours and help to differentiate from other types of paediatric malignant bone and soft-tissue tumours.
描述儿童外周原始神经外胚层肿瘤（pPNETs）的临床、CT及病理表现，以提高对这些罕见肿瘤的认识。
回顾性分析18例经活检或手术病理确诊的儿童pPNETs患者的临床、CT及病理表现。
18例儿童pPNETs患者年龄4个月至15岁，平均年龄7.7岁。病变部位：头颈部4例，胸部2例，腹腔及盆腔6例，脊柱3例，髂骨2例，股骨1例。免疫组化检查显示7个病灶有霍奇金-赖特玫瑰花结，94.4%的病灶CD99染色呈一致性阳性。平扫CT图像上，大多数pPNETs表现为边界不清（72.2%）、形态不规则（83.3%）、密度不均匀（66.7%）或低密度肿块（94.4%），起源于骨骼时伴有溶骨性骨质破坏。3个病灶可见钙化。增强扫描后，所有软组织肿块均呈持续性不均匀强化，内见不同程度的囊变或坏死区，71.4%可见线状强化。94.4%的软组织肿块呈中度强化。7例诊断时已有淋巴结转移。
儿童pPNET可累及身体任何部位，CT图像上表现为边界不清、较大的侵袭性软组织肿块，呈中度不均匀强化，内见不同程度的囊变区及线状强化，伴或不伴有溶骨性骨质破坏，可提示诊断。
原始神经外胚层肿瘤是一种罕见的恶性神经外胚层肿瘤，具有与尤因肉瘤相同的染色体易位，关于儿童该疾病放射学特征的报道不足。本研究描述了18例不同部位诊断为pPNETs的儿童患者的临床特征、CT及病理表现，以提高对这些肿瘤的认识，并有助于与其他类型的儿童恶性骨和软组织肿瘤相鉴别。
