18 LIVER HISTOPATHOLOGY IN PATIENTS WITH HEPATIC MASSES AND THE ABERNETHY MALFORMATION
Annika Nilsen1, Caroline Lemoine2,1, Katherine Brandt2, Saeed Mohammad2,1, Hector Melin-Aldana2,1, Riccardo Superina2,1
1Northwestern University Feinberg School of Medicine, Chicago, USA. 2Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, USA

Abstract

Introduction: To compare the histology of liver biopsies in children with the Abernethy malformation (AM) in the presence or absence of a simultaneous liver mass.

Methods: Liver biopsies in patients with AM between 2004 and 2017 were analyzed retrospectively. Patients were divided into two groups: presence (M+) or absence (M-) of a co-existent liver mass on imaging. Biopsies were reviewed by a pediatric pathologist. Beta catenin stains were done on all biopsies. Chi-square was used for statistical analysis. P values <0.05 were considered significant.

Results: Eighteen of 44 patients had a liver biopsy. Twelve were girls: mean age 3.6± 4.7 years. Thirteen did not have a liver mass. Ten of 13 patients in the M- group and 3 of 5 in the M+ group had a type 1 AM. There was no correlation between presence of mass and type of AM (Type I vs II), p=0.47. All patients had small or absent portal veins. Isolated capillaries outside the portal triad were more frequent in M+ patients (* p=0.017). In contrast, crowding of portal tracts was a feature of M- patients (** p=0.002). Beta catenin staining was negative in all cases. Pathological findings in both groups are compared in Table 1.

Conclusions:

  1. Biopsies associated with AM have vascular and parenchymal abnormalities.
  2. Abnormalities are unrelated to type of malformation.
  3. M+ patients have findings that suggest an increased dependence on arterial blood supply. M- patients demonstrate lobular collapse.
 

M+(n=5)

M-(n=13)

Dilated lymphatics

60% (3/5)

84% (11/13)

Isolated capillaries*

60% (3/5)

8% (1/13)

Focal proliferation of capillaries

40% (2/5)

46% (6/13)

Thickened large arterioles

60% (3/5)

63% (7/11)

Crowding of portal tracts**

0% (0/5)

82% (9/11)

Crowding of central veins

75% (3/4)

90% (9/10)

Portal fibrosis

0% (0/5)

31% (4/13)

Biliary obstructive changes

0% (0/5)

15% (2/13)


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