Niger J Paed 2014; 41 (4): 383 - 385

CASE REPORT

Atimati AO

Hepatoblastoma in an

Abiodun PO

Obaseki DE

adolescent girl: A case report

Olubor OO

DOI:http://dx.doi.org/10.4314/njp.v41i4,18

Accepted: 24th July 2014

Abstract Hepatoblastoma is the

associated with a poor prognosis.

most common primary malignant

We report the case of a sixteen

Atimati AO (

)

hepatic tumour in children, occur-

year old girl who presented with

Abiodun PO

abdominal pain and distension and

Department of Child Health

ring between the ages of 6months

to 3years. It most often presents

jaundice of a short duration. She

Obaseki DE

with a painless abdominal mass

was well-nourished with marked

Department of Pathology

discovered accidentally in young

hepatomegaly and ascites. Hepatic

College of Medical Sciences,

children. Occurrence in adoles-

transaminases were highly ele-

University of Benin,

cents and adults is rare and is usu-

vated with deranged clotting pro-

PMB 1154,

ally associated with non-specific

file. She developed features of

Benin City, Nigeria

symptoms which often result in

hepatic encephalopathy and died

Email: tonyatimati@yahoo.com

delayed diagnosis and commence-

on the seventh day of admission. A

Post-mortem histologic diagnosis

Olubor OO

ment of treatment. Abdominal

Department of Child Health,

pain preceding a rapidly progres-

of hepatoblastoma was made.

University of Benin Teaching Hospital,

sive abdominal mass is a common

Benin City,

pattern observed in adolescents

Key words: Hepatoblastoma,

Nigeria.

and adults. The histologic type

adolescent, poor prognosis

commonly seen in adults is

Introduction

There is paucity of literature on hepatoblastoma in Nige-

ria. A report of primary malignant tumours of the liver

Hepatoblastoma is a rare malignant tumour originating

in Enugu from 1999 – 2005 showed no case of hepato-

blastoma out of 424 patients reviewed . Hepatoblastoma

5

in the cells of the liver . It is the most common primary

1

malignant tumour of the liver in children accounting for

is rare in adolescents and adults and when it occurs, the

symptoms are usually non-specific . Symptoms associ-

6

about 79% of liver cancer in children in the United-

States . Hepatoblastoma has an annual global incidence

2

ated with adult hepatoblastoma in a review of literature

by Zheng et al include failure to thrive, rapidly expand-

6

of 0.5 – 1.5 per million in the paediatric population . A

3

review of 274 children with primary malignant hepatic

ing upper abdominal mass, abdominal pain, vomiting

tumours in South Africa from 1988 – 2006 showed a

and fever. In two other case reports abdominal pain

48% prevalence of hepatoblastoma, 27% hepatocellular

which preceded a rapidly expanding upper abdominal

mass was the main presentation .

7,8

carcinoma while vascular tumours, liver sarcomas and

endodermal sinus tumours constituted 25% . The inci-

4

We present a case of a sixteen year old girl who

dence of hepatoblastoma is highest in infants and falls

presented with a short history of abdominal pain and

off rapidly, with most cases occurring prior to five

swelling and died a week later.

years . There is a higher male to female ratio and white

2

children are often more affected than Negroed children.

1

Case

The aetiology of hepatoblastoma is not quite known, but

cytogenetic abnormalities which include gain of chro-

GL was a sixteen year old girl referred to our hospital

mosome 2, 8, 20, decreased expression of the adenoma-

with complaints of abdominal pain of 9days and ab-

tous polyposis coli gene and increased expression of β -

dominal swelling of 8days duration. The pain was dull

catenin have been described in hepatoblastoma . In-

2

aching, located in the right hypochondrium, severe and

creased incidence has been reported in Beckwith-

associated with backache. Pain was present most of the

Weidemann syndrome, hemi-hypertrophy, familial ade-

time. A day after the onset of abdominal pain, she no-

nomatosis polypi and in children with low birth weight .

2

ticed a swelling in the right hypochondrium which was

Infants and younger children with hepatoblastoma typi-

small initially, but progressively increased in size, ex-

cally present with an asymptomatic abdominal mass,

tending to the epigastric and right lumbar regions. There

and diagnosis is often made late, when the disease is

was associated early satiety. There was no fever or vom-

metastatic . The tumour is mainly unifocal, affecting the

3

iting. Patient’s father and mother died 16years and

right lobe more commonly than the left, and occasion-

3years earlier, respectively, from an unknown cause.

ally affects both lobes. Less common features of hepato-

Examination revealed a well-nourished (weight 59kg,

blastoma are weight loss, anorexia and pain .

2

height 1.52m) female adolescent, who was slightly

384

jaundiced, conscious and well-oriented. The abdomen

with accidental palpation of the liver by caregiver or

was distended with an abdominal girth of 77cm. The

during routine medical examination.Abdominal pain,

liver was palpable 16cm below the right costal margin

which is a prominent symptom in adolescents and

with a span of 26cm. The liver was tender, firm, with a

adults, usually precedes a rapidly expanding abdominal

mass

7,8,10

smooth surface and well-defined edge. There was mod-

. Other symptoms include abdominal disten-

erate ascites demonstrable by shifting dullness. She was

sion, weight loss, anorexia, vomiting and fever. Our

tachynoeic and dyspnoeic with normal percussion notes

patient presented with abdominal pain which was

and breath sounds.

followed shortly by abdominal distension which pro-

Investigations done included: HBsAg (positive), HCV

gressively increased in size.

(negative), prolonged Prothrombin time and Activated

partial thromboplastin time; Total Serum bilirubin

The diagnosis of hepatoblastoma is usually made by

3.5mg/dl, conjugated 1.6mg/dl; normal chest radio-

histology of liver biopsy. Supportive investigations in-

graph; abdominal ultra-sonogram which showed mark-

clude assay for α -fetoprotein, abdominal ultrasonogra-

edly enlarged liver with heterogenous parenchymal

phy, computerized tomography and magnetic resonance

echotexture, smooth outline without a focal mass lesion.

imaging. We did not assay for α -fetoprotein and the diag-

LFT revealed markedly elevated ALP (689IU/L), AST

nosis was missed by abdominal ultrasonography as has

been similarly reported by Al-jiffry in Saudi Arabia

6

(856IU/L), and ALT (294IU/L).

where the diagnosis was missed both with Ultrasound

Fig 1a: (H&E X4) and 1b (H&E X40) shows sheets and rib-

and Computerized Tomography. The tumour is seen as a

bons of small ‘embryonal’ type cells with high nucleo-

hyperechoic, solid, intra-hepatic mass on abdominal

cytoplasmic ratio, nuclear hyperchromasia and small amount

ultrasound . There is no uniform report on the levels of

11

of basophilic cytoplasm.

hepatic transaminases in patients with hepatoblastoma.

The markedly elevated hepatic transaminases in this

patient are at variant with the case reported by Al-Jiffry

with normal levels. This difference might be due to the

stage of the disease which is usually done at the time of

surgery. We, however, did not have the benefit of stag-

ing this patient’s disease since the relatives objected to

autopsy. Inagaki et al similarly reported slightly elevated

hepatic transaminases in an eighteen year old male adult

in Japan .

10

A diagnosis of Fulminant hepatic failure with a differen-

tial of Hepatocellular Carcinoma was made. She was

A positive Hepatitis B surface antigen test in this patient

commenced on vitamins A, D, E and K, Neomycin, lac-

is an unusual finding in Hepatoblastoma. Hepatoblas-

tulose and Dextrose infusion. She also received fresh

toma as often seen in children occurs almost always in

frozen plasma with a view to doing a liver biopsy. Pa-

patients with no underlying liver pathology . This is,

12

tient continued to deteriorate becoming more dyspnoeic

however, different in cases reported in adults where

and restless, with increasing abdominal girth, progres-

there could be co-infection with Hepatitis B as in this

sion of pedal oedema, deterioration of her mental state

patient

3,10

. Similarly, fibrosis or cirrhosis has been ob-

and worsening clotting profile. She died on the seventh

served in adult cases of hepatoblastoma . This has led

3,6

day on admission. A post-mortem needle biopsy was

to the consideration that hepatoblastoma may have a

done since relatives did not consent to an autopsy. His-

different pathogenetic pathway in adults compared to

tologic sections of the liver revealed a malignant neo-

children .

6

plastic lesion composed of dis-cohesive sheets of fairly

uniform small cells with scant cytoplasm.

The cells

The diagnosis of hepatoblastoma was not a strong con-

have oval hyperchromatic nuclei with prominent nucle-

sideration in this patient until demise. In almost all of

oli with presence of pseudorosettes which are consistent

the cases reviewed by Zheng et al, the diagnosis of

4

with embryonal histologic type of hepatoblastoma.

hepatoblastoma was not made until histology of the liver

biopsy was received. This is usually due to the older age

of the patients in which hepatoblastoma is uncommon

and the unusual mode of presentation. In this patient, the

Discussion

tender hepatomegaly, jaundice, ascites, markedly de-

ranged liver enzymes, prolonged prothrombin and par-

Hepatoblastoma usually occurs between the ages of

tial thromboplastin time, positive HBsAg, short duration

6months to 3years and the median age at diagnosis is 1

of symptoms in a well-nourished adolescent led to the

year. Most cases of hepatoblastoma occur before the

9

consideration of fulminant hepatic failure from hepatitis

age of 5years . Therefore, the occurrence of hepatoblas-

2

B virus infection as our initial diagnosis.

toma in this 16year old girl is a very rare occurrence.

Hepatoblastoma is classified by histology as epithelial

Hepatoblastoma has been diagnosed only in a few adults

(56%) or mixed epithelial/mesenchymal (44%). Epithe-

as shown by a review of all published data and library

lial hepatoblastoma is further divided into pure fetal,

search .

3,6

embryonal, macrotrabecular and small cell undifferenti-

ated types . The embryonal histologic type of

2

Infants and younger children may be asymptomatic ,

3

385

hepatoblastoma found in this patient is usually associ-

Conclusion

ated with a poor prognosis . The purely fetal type

13

which has a

favourable prognosis is rarely found in

Hepatoblastoma is a rare hepatic tumour in adolescents

adult who commonly have the mixed histologic type of

and adults. It often presents with asymptomatic abdomi-

hepatoblastoma . Other prognostic factors outside the

3,6

nal mass in younger children. Abdominal pain is a

histologic type include multiple lobes involvement, de-

prominent feature in adults and usually precedes a

creased p27 gene expression, multi-focal dissemination,

rapidly progressive abdominal mass which often has a

AFP( α -fetoprotein) less than 100 or more than

poor outcome. A high index of suspicion is, therefore,

100,000ng/ml , in addition to late presentation and

14

required to make the diagnosis and institute early treat-

mis-diagnosis which might lead to late institution of

ment.

appropriate treatment. These parameters could not be

evaluated in this patient which, could have contributed

Conflict of interest: None

in addition to the poor histologic type to the poor prog-

Funding: None

nosis. Complete surgical resection is the mainstay of

treatment of patients with hepatoblastoma. Administra-

tion of chemotherapy enhances complete excision of un-

resectable hepatic tumours

15

which would have been

offered to our patient had the diagnosis been made.

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