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29/05/2019
Radiological appearances and clinical follow-up of
focal nodular hyperplasia in children
Dr G Chambers, Dr A Zarfati, Prof S Branchereau and Prof Franchi-Abella
What is FNH?
A rare hepatic tumour accounting for approximately 1-2% of all paediatric hepatic
tumours (cf 8% in adults1)
Histologically comprised of nodules of hyperplastic parenchyma with anomalous
organization.
Exact aetiology unknown but maybe due to local blood flow disturbances from micro
vascular disorders1
Natural history of FNH
No malignant potential
Symptomatic
⚫ Abdominal pain
⚫ Compression of organs and vascular structures
Requires differentiation from more aggressive or
problematic tumours e.g. adenoma, Fibrolamellar
HCC
Diagnosing FNH
Imaging
⚫ If typical, diagnosis can be confidently made
⚫ If atypical, may require biopsy or resection
T1 pre Arterial PV Delayed
Aims of our study
Document the imaging features of a cohort of paediatric patients with FNH
Determine the prevalence of typical and atypical features
Describe the natural evolution of these tumours, stratified by intervention
Propose a management algorithm incorporating clinical, imaging and surgical data
Imaging :
Clinical :
Population
• 1970 – 2018
• 88 patients
• 110 lesions
• 1996 – 2018
• 50 patients
• 62 lesions
Imaging cohort Surgical sub-cohort
COHORT DEMOGRAPHICS
Cohort demographics
*4 x CPSS, 2 x malignancy, 1 x cavernoma, 1 x cutaneous vascular malf, 1 x BA w/ PHTN
Child Population Adult Population 1-4
Median age (range) 8 years (6 months – 15 yrs) 38 years (20 yrs - 50 yrs)
Male:Female 1:2 1:9
Median lesion size (range) 5.8cm (7mm – 29cm) 3 cm (1mm – 19cm)
Lesions > 3cm 75% 20 - 50%
Symptomatic 46% 20%
Multiplicity* 11.4% 20%
Population Comorbidities
Other isolated co-morbidities in 9 others
13 patients with vascular
anomalies
4 patients with history of
treated malignancy
3 patients with Sickle
Cell Disease
IMAGING FINDINGS
Well delineated (86%)
Homogeneous (71%)
Iso- or hyperechoic (83%)
Arterial trace (74%)
Scar rarely visible (12.5%)
Ultrasound (89 lesions)
Typical CEUS pattern (n = 7)
Microbubbles are intravascular contrast agents
Have high temporal resolution
Delineate central arterial feeder
Spoke-wheel appearance
8s 10s
20s 28s
Well delineated (78%)
Homogeneous (75%)
Iso- or hypoechoic (95%)
Scar present (55%)Typical tumour enhancement
(72%)
CT (50 lesions)
Hypodense (100%)
Typical scar enhancement
(36%)*
Well delineated (90%)
Homogeneous (78%)
T1 Iso- or hypointense (86%)
Scar present (48%)
MRI (50 lesions)
T2 Iso-hyperintense (100%)
Iso- or hyperintense diffusion
(100%)
T2 Iso- or hyperintense (88%)
Restricted diffusion (0%)
T1 pre Arterial
PV Delayed
Typical tumour enhancement
(68%)
Typical scar enhancement
(86%)
MRI (50 lesions)
Atypical enhancement
23 lesions in 14 patients showed atypical
enhancement
17 lesions in 8 patients with vascular shunts.
Poor arterial enhancement should prompt a
search for a vascular shunt
12 patients had both CT and MR, with no
discordant results
Conclusions - imaging
If a lesion shows typical characteristics, a confident diagnosis of FNH can be made.
If there is atypical arterial enhancement then further histological proof may be required
before management.
Poor arterial enhancement should prompt a search for a vascular shunt
No benefit in repeating cross-sectional imaging if a good quality study.
TREATMENT AND CLINICAL FOLLOW UP
• 1996 – 2018
• 50 patients
• 62 lesions
Surgical sub-cohort
Signs and symptoms – 50 patients
Incidental finding in 27 (54%)
23 (46%) symptomatic at diagnosis
⚫ 15 abdominal pain
Abnormal liver function tests in 18 patients (36%)
⚫ AFP normal
Diagnosis
38 (76%) patients diagnosed confidently by imaging alone
12 (24%) required biopsy
⚫ 11 US-guided
⚫ 1 surgical
Treatment strategy
Indications :
⚫ Symptoms
⚫ Lesion size
Active surveillance First line surgery
⚫ Vascular anomaly
⚫ Uncertain diagnosis
VS
Active surveillance
37 patients (74%) for a mean period of 4.6 years
6 patients (16.2%) had lesion stability
25 patients (67.5%) had lesion growth
6 patients (16.2%) had lesion decrease
Active surveillance
10 patients (27%) required eventual radiological/surgical intervention
Shunt closure :
⚫ 1 x surgical with complete resolution
⚫ 1 x radiological with complete resolution
Resection for :
⚫ 5 x significant lesional growth (including 1 x radiological HA embolisation)
⚫ 3 x intractable symptoms
At the end of follow up : mild abdominal pain (2) and mild dyspnoea (1)
Primary surgical intervention
13 patients (26%) underwent resection
⚫ 5 x intractable symptoms
⚫ 4 x lesion size (mean 12cm)
⚫ 2 x with congenital shunt closure
⚫ 2 x difficult diagnosis
Mean hospital stay was 10.2 days
Only 1 symptomatic at follow up – not thought to be FNH-related
Conclusions – clinical follow up
Lesions likely to increase in size over time
Surgery is effective but should be reserved for patients with :
⚫ Intractable symptoms
⚫ Diagnostic difficulty
⚫ Large lesions with organ/vascular compromise.
No adverse events occurred in the active surveillance group over 4.6 years.
Closure of CPSS resulted in resolution of FNH
Follow up algorithm
Follow up algorithm
Follow up algorithm
Take home messages
Demographic distribution varies significantly
Paediatric FNH share the same imaging characteristics as adults, but:
⚫ Larger lesions (more symptomatic)
⚫ More atypical enhancement (look for a shunt)
⚫ Often multiple – especially after malignancy
⚫ More likely to grow
Active surveillance is a safe and effective first line approach
Surgery should be reserved for difficult diagnosis, intractable symptoms and/or major
solid organ/vascular compression
References
1. Venturi et al. Diagnosis and management of hepatic focal nodular hyperplasia. J
Ultrasound. 2007 Sep; 10(3): 116–127.
2. Geller and Campos. Focal nodular hyperplasia of the liver. Autops Case Rep. 2014
Oct-Dec; 4(4): 5–8.
3. Nguyen et al. Focal Nodular Hyperplasia of the Liver: A Comprehensive Pathologic
Study: 305 Lesions and Recognition of New Histologic Forms
4. Am J Surg Path. 1999 23(12): 1441.
5. Brancatelli et al. Focal nodular hyperplasia: CT findings with emphasis on multiphasic
helical CT in 78 patients. Radiology. 2001; 219:61-68.
THANK YOU FOR YOUR ATTENTION