Benign liver tumours
Key points
A third of liver tumours in children are benign
Three classes of liver tumours
Mesenchymal: Hamartomas and Haemangiomas
Epithelial: Focal nodular hyperplasia and adenomas
Other: Inflammatory pseudotumour, hepatic teratoma
Haemangiomas (Infantile Hepatic Haemangio-endotheliomas (IHHE))
Three types:
Focal (GLUT 1 -ve)
Multifocal (infantile - GLUT 1 +ve)
Diffuse (infantile - GLUT 1 +ve)
Multifocal can evolve into diffuse disease
Diffuse type can potentially replace entire liver parenchyma
Typically presents in infants aged 3-6 months
Associated with Beckwith-Wiedemann syndrome (BWS) and PHACES
Presentation
Anatomical presentations:
Hepatomegaly, abdominal mass, pain, compartment syndrome, cutaneous haemangiomas
Functional presentations:
Bleeding, haemolytic anaemia, jaundice, heart failure, consumptive thrombocytopenia, consumptive hypothyroidism (tumour expression of iodothyronine deiodinase, which inactivates T3/T4)
Rare presentations:
Ascites, feeding difficulties, GI bleeding, rupture, malignant transformation, respiratory compromise
Investigations
Use of Ultrasound (USS) and MRI
Biopsy not required
Focal type has central necrosis and a denser periphery than the liver, distinguishing it from hepatoblastoma
Calcifications appear with involution
Multifocal and diffuse types show well-defined spherical lesions
Course/Progression
Most show rapid growth followed by slow resolution
Treatment
Medical therapy:
Propranolol (1st line)
Steroids
Vincristine (salvage option)
Alpha interferon
Sirolimus
Surgical Management:
Laparostomy
Hepatic artery embolisation or surgical ligation
Liver resection, Liver transplant
Hamartomas
Occur in children under 2 years old
75% found in the right lobe of the liver
3 Types: Hepatic, Biliary, Vascular
Form cysts
Important differential diagnosis: Hydatid disease of liver (requires serology, blood cultures)
Most cases are asymptomatic and discovered incidentally
Likely to involute naturally
Favourable prognosis if diagnosed managed appropriately
Symptomatic hamartomas require excision
Large hamartomas can cause heart failure, hydrops, respiratory distress, or death
Focal Nodular Hyperplasia
Rare liver tumour (solid benign mass of non-vascular origin)
Increased hepatocyte mass due to hypo/hyper perfusion from anomalous arteries in the hepatic lobule (arterial malformations)
Identified by a dense central stellate scar, best seen on CT
Unlikely to become malignant
Managed conservatively
Resection if symptomatic or diagnostic uncertainty
Hepatic Adenomas
Common in adolescents, especially with used of combined oral contraceptive pill and young men using anabolic steroids
Associated with glycogen storage diseases (Type I and III), obesity, metabolic syndrome, diabetes mellitus
Variable appearance on USS: Hyper or hypoechoic, fatty, haemorrhagic necrosis
Well-circumscribed lesion on CT
May require resection especially if larger than 5 cm
Other treatment modalities: Conservative, embolisation, radio-frequency ablation
Inflammatory Myofibroblastic Tumours
Characterised by spindle cell proliferation with chronic inflammatory infiltrate
Benign but locally aggressive
50% have rearrangement of the anaplastic lymphoma kinase gene, treatable with crizotinib
Primary treatment is excision
Page edited by Prof. Ashok Daya Ram, Consultant Paediatric and Neonatal Surgeon, Norfolk and Norwich University Hospital, UK. November 2024
References
McEachron, Kendall, et al. "Benign Liver Tumors." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829481/all/Benign_Liver_Tumors.
Shreenath AP, Grant LM, Kahloon A. Hepatocellular Adenoma. [Updated 2024 May 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513264/
Hamad S, Willyard CE, Mukherjee S. Focal Nodular Hyperplasia. [Updated 2022 Sep 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK532244/