Ma Irene T, Rojas Yesenia, Masand Prakash M, Castro Eumenia C, Himes Ryan W, Kim Eugene S, Goss John A, Nuchtern Jed G, Finegold Milton J, Thompson Patrick A, Vasudevan Sanjeev A
Department of Surgery, Mayo Clinic Arizona, Phoenix, Arizona.
Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas.
J Pediatr Surg. 2015 Mar;50(3):382-7. doi: 10.1016/j.jpedsurg.2014.06.016. Epub 2014 Aug 27.
Focal nodular hyperplasia (FNH) is uncommonly diagnosed in pediatric patients and may be difficult to distinguish from a malignancy. We present a review of all children with a tissue diagnosis of FNH at our institution, describe the diagnostic modalities, and provide recommendations for diagnosis and follow-up based on our experience and review of the literature.
A retrospective review of children <18years of age diagnosed with FNH at a single institution was performed from 2000 to 2013.
Twelve patients were identified with a tissue diagnosis of FNH. Two patients required surgical resection of their lesion owing to concern for malignancy. Ten patients were managed expectantly with imaging surveillance after biopsy confirmed a diagnosis of FNH. All patients who underwent MRI had very typical findings including hypointensity on T1 weighted sequences, hyperintensity on T2, and homogenous uptake of contrast on the arterial phase. On follow-up all patients had either a stable lesion or reduction in size.
Focal nodular hyperplasia presents typically in children with liver disease, have undergone chemotherapy, and adolescent females. Young children, particularly <5years of age, without underlying liver disease or history of chemotherapy can pose a diagnostic dilemma. In this unique subgroup of children with FNH, MRI and/or needle biopsy should be adequate diagnostic modalities for these lesions.
局灶性结节性增生(FNH)在儿科患者中罕见诊断,且可能难以与恶性肿瘤区分。我们对本机构所有经组织学诊断为FNH的儿童进行了回顾,描述了诊断方法,并根据我们的经验和文献回顾提供诊断及随访建议。
对2000年至2013年在单一机构诊断为FNH的18岁以下儿童进行回顾性研究。
确定12例患者经组织学诊断为FNH。2例患者因怀疑恶性肿瘤而接受了病变的手术切除。10例患者在活检确诊为FNH后接受影像学监测。所有接受MRI检查的患者均有非常典型的表现,包括T1加权序列低信号、T2加权序列高信号以及动脉期造影剂均匀摄取。随访中,所有患者的病变均稳定或缩小。
局灶性结节性增生通常见于患有肝病、接受过化疗的儿童以及青春期女性。没有潜在肝病或化疗史的幼儿,尤其是5岁以下的幼儿,可能会造成诊断难题。在这个FNH儿童的独特亚组中,MRI和/或穿刺活检应该是这些病变足够的诊断方法。