radiological appearances and clinical follow-up of focal nodular hyperplasia … · 2019-05-29 ·...
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Direction
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
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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
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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
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Diagnosing FNH
Imaging
⚫ If typical, diagnosis can be confidently made
⚫ If atypical, may require biopsy or resection
T1 pre Arterial PV Delayed
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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 :
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Population
• 1970 – 2018
• 88 patients
• 110 lesions
• 1996 – 2018
• 50 patients
• 62 lesions
Imaging cohort Surgical sub-cohort
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COHORT DEMOGRAPHICS
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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%
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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
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IMAGING FINDINGS
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Well delineated (86%)
Homogeneous (71%)
Iso- or hyperechoic (83%)
Arterial trace (74%)
Scar rarely visible (12.5%)
Ultrasound (89 lesions)
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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
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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%)*
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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%)
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T1 pre Arterial
PV Delayed
Typical tumour enhancement
(68%)
Typical scar enhancement
(86%)
MRI (50 lesions)
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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
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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.
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TREATMENT AND CLINICAL FOLLOW UP
• 1996 – 2018
• 50 patients
• 62 lesions
Surgical sub-cohort
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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
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Diagnosis
38 (76%) patients diagnosed confidently by imaging alone
12 (24%) required biopsy
⚫ 11 US-guided
⚫ 1 surgical
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Treatment strategy
Indications :
⚫ Symptoms
⚫ Lesion size
Active surveillance First line surgery
⚫ Vascular anomaly
⚫ Uncertain diagnosis
VS
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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
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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)
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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
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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
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Follow up algorithm
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Follow up algorithm
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Follow up algorithm
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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
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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.
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