Chambers Greg, Zarfati Angelo, Aderotimi Tobi, Branchereau Sophie, Humphrey Terry, Woodley Helen, Franchi-Abella Stéphanie
Department of Paediatric Radiology, Leeds General Infirmary, Great George Street, LS1 3EX, Leeds, UK.
Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Rome, Italy.
Pediatr Radiol. 2023 Jan;53(1):46-56. doi: 10.1007/s00247-022-05420-9. Epub 2022 Jul 1.
Focal nodular hyperplasia (FNH) in children is a rare but benign tumour, which must be differentiated from malignant entities to avoid unnecessary treatment, leading to potential morbidity.
To provide data on imaging findings of these lesions with a suggested algorithm for diagnosis, sampling and follow-up.
This retrospective review evaluated imaging of all patients diagnosed with FNH in two tertiary referral centres in Europe between 1975 and 2018.
One hundred and four patients with 137 tumours were reviewed. The mean age at presentation was 8.2 years. The median tumour size was 5 cm (range: 0.3-29 cm). Multiple lesions were seen in 16.3% of patients. The male-to-female ratio was 1:2.
FNH with typical features on imaging can be safely followed up once the diagnosis has been established. The use of contrast-enhanced ultrasound and magnetic resonance imaging allows accurate characterisation in most cases. Histological sampling is only advised when there is diagnostic doubt. Atypical arterial enhancement of FNH should prompt the search for a congenital portosystemic shunt.
儿童局灶性结节性增生(FNH)是一种罕见的良性肿瘤,必须与恶性病变相鉴别,以避免不必要的治疗及其潜在的发病率。
提供这些病变的影像学表现数据,并提出诊断、取样和随访的算法。
本回顾性研究评估了1975年至2018年间欧洲两家三级转诊中心所有诊断为FNH的患者的影像学资料。
共回顾了104例患者的137个肿瘤。就诊时的平均年龄为8.2岁。肿瘤中位大小为5 cm(范围:0.3 - 29 cm)。16.3%的患者有多个病灶。男女比例为1:2。
一旦确诊,具有典型影像学特征的FNH可安全随访。在大多数情况下,使用对比增强超声和磁共振成像可进行准确的特征描述。仅在诊断存疑时建议进行组织学取样。FNH的非典型动脉强化应促使寻找先天性门体分流。