radiological appearances and clinical follow-up of focal nodular hyperplasia … · 2019-05-29 ·...

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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