Management of focal nodular hyperplasia of the liver: Experience of 50 pediatric patients in a tertiary center

Background: Focal nodular hyperplasia (FNH) is a rare benign hepatic lesion in children. No management guidelines for pediatric population exist because of limited evidence.

Objective: To review the experience of a large tertiary liver center, providing additional clinical data to help formulate management guidelines for FNH in the pediatric population.

Methods: We analyzed data of children <18 years diagnosed with FNH from 1996 to 2018 at our hospital, detailing management and long-term clinical outcome.

Results: 50 patients were identified. The median age was 10 years old (range 0.75-15.5 years old). The mean diameter of FNH was 5.9 cm (±3.1 cm). 10 patients had multiple lesions. First-line management: watchful waiting with serial checks (n = 37), surgery (n = 13). Of the watchful waiting patients, 10 required eventual second-line surgery. After a median follow-up of 4.7 years (range 0.5-20 years), 46 patients were asymptomatic, with no significant difference in clinical outcome (p = 0.962) between the two first-line management approaches. Lesions demonstrated growth in 13 cases: 5 of these required second-line surgery. In these patients, there was no significant difference in clinical outcome (p = 0.188) compared to nonoperative patients. Considering all surgically treated patients, there was no significant difference between first-line and second-line surgery for clinical outcome (p = 0.846), hospital stay (p = 0.410), complications (p = 0.510) and severe complications (p = 0.385).

Conclusions: Our data support the hypothesis that watchful waiting is a safe initial approach to pediatric FNH management in patients with no major symptoms or complications. Surgery should be reserved for patients with diagnostic doubt, persistent symptoms and/or biological or significant anatomical abnormalities. FNH growth alone should not be considered as an indication for surgery.

Type of study: Therapeutic study.

Level of evidence: Level III.