Elsevier

Journal of Pediatric Surgery

Volume 45, Issue 9, September 2010, Pages 1797-1803
Journal of Pediatric Surgery

Original article
Focal nodular hyperplasia in children: clinical features and current management practice

https://doi.org/10.1016/j.jpedsurg.2009.12.027Get rights and content

Abstract

Background

Although nonoperative management is an accepted practice for most adults with focal nodular hyperplasia (FNH), questions remain about the safety and feasibility of this strategy in children. Our aim was to review the clinical features of children with FNH and determine current management patterns.

Methods

We reviewed records of all children and adolescents with FNH managed at our institution from 1999 to 2009 and performed a MEDLINE search to identify all published cases of FNH in the pediatric population.

Results

A total of 172 patients with FNH were identified, including 11 at our institution. The median age at diagnosis was 8.7 years and 66% were female. Median tumor size was 6 cm, and 25% had multiple lesions. Thirty-six percent were symptomatic at presentation. Twenty-four percent had a history of malignancy. Management included resection (61%), biopsy followed by observation (21%), and observation alone (18%). Indications for resection included symptoms (48%), inability to rule out malignancy (24%), tumor growth (15%), and biopsy-proven concurrent malignancy (9%).

Conclusions

Although FNH is a benign lesion that is typically managed nonoperatively in adults, most children with FNH currently undergo resection because of symptoms, increasing size, or inability to confidently rule out malignancy.

Section snippets

Materials and methods

Inpatient and outpatient hospital records were reviewed in all patients identified as having FNH. Clinicopathologic data reviewed included case histories, laboratory results, operative reports, imaging results, pathology reports, and follow-up examinations. The protocol was approved by the Institutional Review Board of Children's Memorial Hospital (Chicago, IL).

The MEDLINE database was reviewed for the period January 1980 to January 2009 to identify pediatric and adolescent cases of FNH.

Institutional experience

Eleven patients with histologically proven FNH were identified between 1999 and 2009 at our institution. The median age at diagnosis was 12.8 years (range, 1-20 years) and 8 were female. Three patients were symptomatic at presentation, whereas 6 were discovered incidentally on computed tomographic (CT) imaging, 1 was found incidentally during laparotomy, and 1 was found incidentally on physical examination. Liver function tests were abnormal in 2 and α-fetoprotein was elevated in 1 patient.

Cumulative experience

A

Discussion

Review of our own experience managing 11 children with FNH, combined with 161 additional patients in the literature, demonstrates that nonoperative management is feasible in an appropriately selected subset of children and adolescents. However, 60% to 80% currently undergo tumor resection because of symptoms, increasing size, or inability to confidently rule out malignancy. Focal nodular hyperplasia occurs in any age group, and approximately 66% of children with FNH are female, compared to a

References (47)

  • IshakK.G. et al.

    Benign tumors of the liver

    Med Clin North Am

    (1975)
  • WeimannA. et al.

    Benign liver tumors: differential diagnosis and indications for surgery

    World J Surg

    (1997)
  • OttR. et al.

    Focal nodular hyperplasia and liver cell adenoma: operation or observation

    Zentralbl Chir

    (1998)
  • ColliA. et al.

    Elective surgery for benign liver tumours

    Cochrane Database Syst Rev

    (2007)
  • MuellerB.U. et al.

    Tumors of the liver

  • StockerJ.T. et al.

    Focal nodular hyperplasia of the liver: a study of 21 pediatric cases

    Cancer

    (1981)
  • SudourH. et al.

    Focal nodular hyperplasia of the liver following hematopoietic SCT

    Bone Marrow Transplant

    (2009)
  • YangY. et al.

    Management and surgical treatment for focal nodular hyperplasia in children

    Pediatr Surg Int

    (2008)
  • FujitaS. et al.

    Liver-occupying focal nodular hyperplasia and adenomatosis associated with intrahepatic portal vein agenesis requiring orthotopic liver transplantation

    Transplantation

    (2006)
  • DemirH.A. et al.

    Focal nodular hyperplasia of the liver and elevated alpha fetoprotein level in an infant with isolated hemihyperplasia

    J Pediatr Hematol Oncol

    (2008)
  • FreidlT. et al.

    Focal nodular hyperplasia in children following treatment of hemato-oncologic diseases

    Klin Padiatr

    (2008)
  • OkugawaY. et al.

    Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy

    Pediatr Surg Int

    (2008)
  • MarabelleA. et al.

    Focal nodular hyperplasia of the liver in patients previously treated for pediatric neoplastic diseases

    J Pediatr Hematol Oncol

    (2008)
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