ttPSurgery, 1995, Vol. 9, pp. 47-49
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CASE REPORT
Hepatobl,astoma in an Adult with Biliary
Obstruction and Associated Portal Venous
Thrombosis
LAV K. KACKER*, E. M. KHAN**, ROHIT GUPTA***, V. K. KAPOOR*, RAKESH PANDEY**,
R. K. GUPTA#, and V. A. SARASWAT***,
*Department of Surgical Gastroenterology,**Department of Pathology,***Department of Gastroenterology, #Department of
Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences; Raebareli Road, Lucknow UP, India
(Received23 May 1993)
We present a case of adult hepatoblastoma. This young female presented with severe acute
cholangitis. Preoperative diagnosis was common bile duct (CBD) obstruction with portal vein
thrombosis. On exploration she had a tumor mass in the CBD. The unusual features ofthis case
are discussed in this report.
KEY WORDS: Hepatoblastoma portal vein thrombosis biliary obstruction
INTRODUCTION
Hepatoblastomas are tumors of children and are rare
in adults. Wereport a case of hepatoblastoma in a 28
year old woman with unusual clinical, morphologic
and histologic features.
CASE REPORT
A 28 year old woman presented with presistent, epigas-
tric and right upper quadrant pain, cholestaticjaundice
and intermittent, low grade fever for 2 months. Exam-
ination revealed anaemia, deep jaundice and pedal
edema. Her temperature was 38.4C. The liver was
palpable 4 cm below the right costal margin and was
tender and smooth. Free fluid was present in the abdo-
men. The gall bladder and spleen were not palpable.
Hemoglobin was 8.2 gm%; WBC count 6000,cells/
cumm with 80% polymorphs; blood sugar and renal
Addressfor correspondence." VA Saraswat, Associate Professor,
Department of Gastroenterology, Sanjay Gandhi Postgraduate
Institute of Medical Sciences, Raibareli Road, Lucknow, India.
47
function were within normal limits; total serum protein
was 4.8 gm% and serum albumin 2.1 gm%; total serum
bilirubin 20. 8 mg% with direct bilirubin 12.0 mg%;
alkaline phosphatase 166 U/L (range 35-170 U/L);
SGOT and SGPT 70 and 12 U/L (range 5-40 U/L)
respectively. Prothrombin time and activated partial
thromboplastin time were marginally prolonged. The
ascitic fluid was transudative and exfoliative cytology
was negative for malignant cells. Upper gastro-intesti-
nal endoscopy did not reveal oesophagogastric varices.
Ultrasonographic examination showed an irregular
liver surface with loss of normal architecture. The gall
bladder was markedly distended with sludge and the
CBD was dilated down to the the lower end with the
echogenicity of the bile similar to that of the gall blad-
der, suggesting sludge in the CBD. Intrahepatic biliary
ducts were dilated in the left lobe while right lobe ducts
could not be visualized. There was no space occupying
lesion in the liver. There was a thrombus in the extrahe-
patic portal vein with periportal and retro-peritoneal
collaterals.
Percutaneous liver biopsy, done at this stage, did
not show any evidence of liver cirrhosis. However,
48 L.K. KACKER et al.
cholestasis, mild bile ductular proliferation and poly-
morphonuclear infiltration suggested extrahepatic
bile duct obstruction with cholangitis. In order to
obtain a better delineation of the anatomy, a CT scan
was performed, which confirmed the sonographic
findings and showed that CT attenuation values of
gall bladder and CBD contents were similar (36 HU).
ERCP showed gross dilatation of the CBD (20 mm)
with a large intra-luminal filling defect (4.5 x 1.5 cm).
During injection, contrast could be seen streaming
around this mass which extended from just above the
papilla to the porta hepatis. The right hepatic duct and
its branches were blocked while the left hepatic duct
was opacified adequately.
To relieve the cholangitis, an endoscopic naso-
biliary drain was placed in the left hepatic duct. This
drained about 50 ml bile per day and bile culture grew
Pseudomonas aeruginosa. Despite appropriate anti-
biotic therapy and nasobiliary drainage, cholangitis
continued and operative decompression of the biliary
system was necessary. The preoperative diagnosis was
liver cirrhosis, probably cryptogenic, with portal hy-
pertension, portal vein thrombosis, extrahepatic bili-
ary obstruction due to CBD sluge or stones and liver
failure. Exploration revealed macronodularity of both
lobes of the liver and ascites. The gall bladder was
distended with sludge and thick viscid bile. Dilated
vessels were present along the hepatoduodenal liga-
ment. The CBD was grossly dilated (3cm) and filled
with fleshy and necrotic tissue which was removed
after choledochotomy. Extra hepatic biliary ducts
were cleared and a T-tube was placed. Cholecystosto-
my was also done. Needle biopsies of the liver were
taken. During the postoperative period the cholangi-
tis settled. T-tube cholangiogram on the 14th postop-
erative day showed a blocked right hepatic duct. The
left ductal system and CBD were dilated but free of
any filling defects and constrast flowed freely into the
duodenum. The patient was discharged after 2 weeks
with the T-tube in situ.
Liver biopsies obtained during surgery showed ex-
tensive parenchymal and canalicular cholestasis and
features of acute cholangiohepatitis. There was no evi-
dence ofliver cirrhosis and no tumor tissue was seen in
the biopsy. Tissue obtained from the CBD consisted of
multiple small, soft, fleshy and necrotic pieces. Micro-
scopically, the tumor was predominantly composed of
sheets, trabecular cords and islands ofpolyhedral cells
separated by sinusoidal channels lined by endothelial
cells. These cells showed slightly anisomorphic, round
to oval nuclei with inconspicuous nucleoli and abun-
dant pale to lightly granular cytoplasm. At places,
islands of small, primitive, embryonal cells were
present with hyperchromatic nuclei and a narrow rim
of eosinophilic cytoplasm. The mesenchymal compo-
nent was seen as irregular bands and large islands of
primitive fibro-myxomatous appearance with plump,
spindle shaped and elongated nuclei. Frequent mitotic
figures (1-3 HPF) were seen. Many of the fragments
were completely necrotic. Histopathologic diagnosis
was hepato-blastoma with cholestasis and cholangio-
hepatitis.
Liver function parameters 4 weeks after surgery did
not show any improvement. Serum bilirubin was
20 mg% with direct fraction of8.9 mg% serum alkaline
phosphatase 132 U/L; SGOT/SGPT 59 and 124 U/L
respectively, total proteins 5.6 gm% with serum albu-
min 2.0 gm%. Serum alfafetoprotein (AFP) level was
9000 IU/ml (normal 0.5-55 IU/ml). The patient was
lost to followup after this hospital visit.
DISCUSSION
This 28 year old woman with hepatoblastoma involv-
ing the intrahepatic and extrahepatic biliary channels
presented with several unusual features.
Hepatoblastoma is very rare in adults. It is primarily
a tumor of young children with 92% of the cases pre-
senting below the age of 5 years1. Only 20 cases ofadult
hepatoblastoma have been reported in the English liter-
ature2-8. Jaundice, as a presenting feature, is uncom-
mon in hepatoblastoma having been noted in less than
6% of cases9. This is the first report of adult hepat-
oblastoma presenting with obstructive jaundice. A va-
riety ofmechanisms are responsible forjaundice in liver
tumors1. These include tumor infiltration into the he-
patic parenchyma, compression of biliary channels by
tumor mass or lymphnodes, tumor necrosis with hemo-
bilia, pedunculated tumor extension, clot or tumor de-
bris within the bile ducts and associated cirrhosis. In all
likelihood, extension ofthe tumor into the right hepatic
duct and the common bile duct from a microscopic
parenchymal focus in the right lobe resulted in biliary
obstruction andjaundice in our patient. The cholangio-
graphic appearance of tumor invading the bile ducts is
described as a smooth or lobulated intraluminal filling
defect, giving rise to the'goblet' or 'wineglass' appear-
ance1. This is identical to the appearance noted in our
patient. Peroperatively tumors are described as fleshy,
soft and compressible, similar to chicken fat11, as was
observed in our patient.
Hepatoblastoma commonly forms a single mass
within the right lobe of the liver1,2; less commonly mul-
tiple nodules may be present, usually in both lobes1,2.
HEPATOBLASTOMA IN AN ADULT 49
The least common is diffuse tumor involving the entire
liver. Cirrhosis is rarely present with hepatoblasto-
ma1,2. In our patient no mass lesion was detected in the
liver on US, CT or at surgery. Diffuse involvement of
the liver was ruled out by negative liver biopsies. This
unusual presentation ofhepatoblastoma with a micro-
scopic parenchymal focus growing primarily into the
extrahepatic biliary ducts has not been reported be-
fore. Possibly, extension of tumor thrombus into the
portal vein may have resulted in portal vein thrombo-
sis, contributing to portal hypertension. The associa-
tion ofthis complication with hepatoblastoma has also
not been reported in the literature. Histologically,
hepatoblastoma is classified into epithelial and mixed
(epithelial and mesenchyml) types. The epithelial com-
ponent may be offetal or embryonal cell type12. There
is a single case report of epithelial hepatoblastoma in
an adult4. All other cases of adult hepatoblastoma are
of mixed type, as was seen in our case.
CONCLUSION
We report a case of hepatoblastoma in a 28 year old
woman. The tumor had invaded the biliary channels
and CBD causingjaundice and cholangitis. Portal vein
thrombosis was also present and liver failure had de-
veloped probably due to the combination of biliary
and portal venous obstruction along with associated
severe cholangitis.
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