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| Journal of the New Zealand Medical Association,
20-April-2012, Vol 125 No 1353
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Hepatocellular carcinoma (HCC) has a distribution that
typically follows the prevalence of the hepatitis B and C viruses. As a
consequence a third of cases are found in China and another third in the rest of
Asia.1
In New Zealand, rates of HCC for Māori and Pacific
people were 7.3 and 18.0 times that for other
ethnicities.2 HCC in pregnancy is extremely
rare, especially if viral hepatitis negative.
We present a case of HCC diagnosed in pregnancy in a young
New Zealand Māori woman in an otherwise normal liver. This case highlights
the difficulty in diagnosis preoperatively, and timing of surgery in the
presence of a viable fetus.
Case reportA 33-year-old, gravida eight para four, Māori woman,
had a liver mass detected on routine prenatal ultrasound scan at 20 weeks
gestation. Her alpha feto protein level was 295 and hepatitis screen was
negative. She had no history of oral contraceptive (OCP) use.
She underwent an MRI scan (Figure 1) which showed a large
(24 cm) lobulated, heterogenous mass centred in segments IVB and V with areas of
restricted diffusion, and heterogenous enhancement including areas of arterial
phase enhancement which showed washout on the venous phases and areas of delayed
enhancement.
Given the clinical setting and MRI appearances, the lesion
was thought most likely to represent a liver cell adenoma (LCA) or HCC. Biopsy
was deemed to be inappropriate as it was unlikely to yield a definite diagnosis
and carried an undue risk of rupture.
The patient was admitted at 30 weeks gestation for lower
segment caesarean section and simultaneous resection of the hepatic mass.
Laparotomy revealed a large, lobulated liver tumour from segments IV, V and VI
(Figure 2). The lesion was successfully resected with good haemostasis and no
bile leak.
Figure 1. Portal venous phase post contrast T1
fat saturated (VIBE) image showing liver mass. The patient was scanned on her
side which explains the contour of her abdomen
 Macroscopically, the tumour had a thick pseudocapsule, it
measured 290 × 180 × 140 mm and weighed 3.7 kg (Figure 2). Microscopic
examination showed a poorly differentiated HCC (Fig. 3). There were sheets or
trabeculae of tumour cells with fibrovascular stroma, focal haemorrhage and
necrosis.
The tumour cells had clear, foamy to eosinophilic cytoplasm.
Some had bizarre multilobated nuclei, and mitotic figures were frequently seen.
There was vascular space invasion, but the resection margins were clear.
Immunohistochemically the tumour cells were moderately positive for HepPar1, and
showed a canalicular staining pattern for CD10. The uninvolved liver showed no
evidence of cirrhosis, chronic hepatitis, or other underlying
abnormalities.
Figure 2. Intraoperative image of resected
hepatic tumour
 Figure 3. Microscopic examination showing
features consistent with poorly differentiated hepatocellular carcinoma (details
in text)
 Postoperatively the patient had a CT chest and abdomen which
showed no residual HCC. There was no indication for further chemotherapy or
radiotherapy. There was no evidence of recurrent disease at 1-year follow up.
DiscussionHepatocellular carcinoma is uncommon in pregnancy. This is
partly because cirrhosis, which predisposes to HCC, is associated with
infertility. However, oral contraceptives, early menarche, late menopause and
increasing parity have all been shown to contribute a small risk to HCC
development, suggesting oestrogens play an important role. There are also
several case reports of LCA transformation into HCC years after cessation of OCP
use.3
With the scarcity of cases it is difficult to quantify the
effects and risks of pregnancy, specifically rupture, accelerated growth and a
poorer prognosis. However, worse outcomes in pregnant women with HCC were noted
in the literature. One review quoted only three of 29 patients surviving 12
months or more and live infants being delivered in only 57% of
cases.4 Another case study followed an HCC over
time and noted acceleration of growth during the
pregnancy.5
A 2011 retrospective review of all 47 case studies worldwide
of HCC in pregnancy showed poor but improving survival rates over time (median
survivals of the groups before and during/after 1995 were 18 and 25.5 months,
respectively).6 Improving survival is due to
both earlier diagnosis and surgical
intervention.6 This need for early imaging and
resection poses an obvious challenge in pregnancy.
Management has traditionally focused on termination due to
the adverse effects of pregnancy on the tumour, followed by resection. This is
often undesirable, such as the present case, where a diagnosis was not made
until 20 weeks gestation.
Resection is undoubtedly the gold standard of management if
possible. However this must be weighed with risks to the fetus of early
delivery. Unfortunately there are no clear guidelines on timing of resection due
to the rarity of the condition and patient variables. Adjunctive measures such
as steroids for fetal lung maturation are recommended to allow earlier
delivery.
We report on an interesting and rare case of a
hepatocellular carcinoma in a young, pregnant female with an otherwise normal
liver. This case highlights the difficulty in making a diagnosis based solely on
imaging. Even in a patient with no known risk factors, early resection remains
the gold standard for management if it is unclear whether the mass is an HCC or
LCA.
Timing of delivery and resection must be balanced between
fetal maturation and increasing risks of rupture and tumour development and
taken on an individual basis.
Author information: Peter Russell, House
Officer; Pandanaboyana Sanjay, HPB Fellow; Peter Johnston, HPB Surgeon,
Department of General Surgery; Ilse Dirkzwager, Radiologist, Department of
Radiology; Kai Chau, Pathologist, Department of Pathology; Auckland City
Hospital, Auckland
Correspondence: Dr Sanjay Pandanaboyana,
Fellow in Hepatobiliary Surgery, Auckland City Hospital, Private Bag 92 024,
Auckland 1142, New Zealand. Email: sanjay.pandanaboyana@nhs.net
References:
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