Adult Hepatoblastoma: a Case Report and Literature Review

CASE REPORT – OPEN ACCESS

International Journal of Surgery Case Reports 4 (2013) 204–207

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International Journal of Surgery Case Reports

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Adult hepatoblastoma: A case report and literature review

a,b,∗

B.O. Al-Jiffry

Department of Surgery, College of Medicine and Medical Sciences, Taif University, PO Box 888, Taif 21947, Kingdom of Saudi Arabia

Department of Surgery, Al-Hada Military Hospital, PO Box 1347, Taif, Kingdom of Saudi Arabia

a r t i c l e i n f o a b s t r a c t

Article history: INTRODUCTION: Adult hepatoblastoma is a rare malignant liver neoplasm. Surgery is the only cure, but

Received 17 August 2012

recurrence is common even after complete resection. No therapeutic strategy has been established.

Received in revised form 5 October 2012

PRESENTATION OF CASE: A 22-year-old man presented with a rapidly expanding right hypochondrial mass.

Accepted 27 October 2012

Pain preceded the appearance of the mass. No deﬁnitive diagnosis was established in the referring hospi-

Available online 24 November 2012

tal. In addition, two attempts of embolization failed to reach the tumor due to its large size and vascular

displacement. Clinical examination revealed a 26 cm 23 cm mass occupying the right hypochondrium

Keywords:

and epigastrium as far as the right iliac fossa, compressing the stomach, spleen, kidneys and liver. The

Hepatoblastoma

preoperative diagnosis was gastrointestinal stromal tumor because it appeared to originate from the

Liver malignancies

stomach. During surgery, we found a mass arising from the liver, adhering to the omentum, stomach, and

Primary liver tumors

Adult left hemidiaphragm, and inﬁltrating the pericardium. The tumor was completely resected off the inferior

vena cava and pericardium. The histopathological diagnosis was a 30 cm × 30 cm hepatoblastoma weigh-

ing 4 kg. The postoperative treatment course went smoothly until day 10, when the patient developed

complications like bilateral atrial thrombi and left ventricular hypokinesia and expired on day 16.

DISCUSSION: Due to the rarity of hepatoblastoma in adults and non-speciﬁc initial symptoms, hepa-

toblastoma is often overlooked as a diagnosis. Early detection may lead to improved prognosis and

survival.

CONCLUSION: We report here the ﬁrst case of adult hepatoblastoma in the Middle East and the largest

such tumor ever reported in literature.

1. Introduction 2. Presentation of case

Hepatoblastoma is the most common liver tumor in children 2.1. Presentation

under the age of 2 years. Reports of adult cases are extremely rare.

Most of these tumors arise in the embryo during liver develop- Our patient was an otherwise healthy 22-year-old Saudi male

ment and have embryonic features such as numerous mitoses, and who was referred to us from another hospital. He had a large and

often present with ribbon-like or papillary formations. In adults, expanding abdominal mass that had been palpable for 6 months.

the prognosis is extremely poor. Although surgery offers the only The patient initially presented with dull aching epigastric and right

chance for cure, it often recurs even after complete resection, and upper quadrant abdominal pain. The referring hospital did not diag-

no therapeutic strategies have yet been established because of its nose the condition, but had attempted embolization with stainless

3,4 3

rarity. A review by Zheng et al. reports only 15 cases in the steel coils to reduce the vascularity and size of the mass; however,

English literature, where nine cases were treated by surgery, two they failed due to the large size of the tumor and vascular displace-

by surgery followed by chemotherapy and one by chemoemboliza- ment. They attempted to go through both the left gastric and the

tion; however, they all had a very poor prognosis. In another more hepatic artery. Upon examination, a large, palpable mass extend-

recent review, Rougemont et al. described a total of 45 cases of ing from the right hypochondrium and epigastric region to the right

adult hepatoblastoma, all with very dismal prognosis due to late iliac fossa was found. There was no lymphadenopathy, and systemic

presentation and with no consensus on the treatment due to the examination showed normal results.

rarity of the disease. Most of these patients expired before surgery

or soon after surgery. We present the ﬁrst case of adult hepatoblas-

toma reported in the Middle East. 2.2. Laboratory investigations

In our hospital, CBC, LFTs, lipase, and amylase levels, and the

∗ results of renal panel were normal. The tumor markers present

Correspondence address: Department of Surgery, College of Medicine and Med-

were CEA (<0.5 ng/mL) and AFP (1.3 ng/mL). Tests for hepatitis

ical Sciences, Taif University, PO Box 888, Taif 21947, Kingdom of Saudi Arabia.

markers (B & C) were negative.

E-mail address: [email protected]

CASE REPORT – OPEN ACCESS

B.O. Al-Jiffry / International Journal of Surgery Case Reports 4 (2013) 204–207 205

2.4. Operative ﬁndings

We decided to conduct exploratory laparotomy and possible

resection of the tumor. After discussion with the patient and

his family on the risks of the procedures, informed consent was

obtained. A midline incision revealed a large mass that was adher-

ent to the omentum, pushing the bowel downward, the liver

and spleen laterally, and the stomach and pancreas posteriorly. It

extended to the chest and involved the left hemidiaphragm and

the pericardial space. The mass was mobilized with good hemosta-

sis. Initially, we thought that it originated from the stomach, so

we opened the stomach and found that the only structure adher-

ent to the stomach was the tumor capsule. Dissection of the mass

showed that it originated from the left hemiliver and was push-

ing on the pericardium and diaphragm. Exposure of the heart via

median sternotomy resulted in a gush of ﬂuid from the pericar-

dial space. En-block excision of the tumor was performed using

a piece of the diaphragm, and a left hepatic lobectomy was per-

formed. The tumor was found to involve a part of the inferior vena

cava (IVC), just inferior to the diaphragm. The tumor was shaved

from the inferior vena cava, and the entire mass was excised and

sent for histopathological examination. The sternotomy wound was

Fig. 1. Large mass seen in the upper abdomen.

closed and good hemostasis was maintained in the chest. At this

point the patient became hypotensive and began to bleed because

of coagulopathy from the long procedure and acidosis, where he

2.3. Radiological investigations

was transfused 11 Units of PRBC and 8 Units of FFP. A decision was

made to pack all the areas around the liver and spleen, and the

Abdominal ultrasonography revealed a hypoechoic lesion

abdomen was closed.

in the epigastric region adjacent to the left liver measur-

ing 24.7 cm 12.8 cm. Contrast-enhanced abdominal computed

2.5. Postoperative care

tomography showed a very large heterogeneous mass of about

26 cm × 23 cm occupying the anterior part of the abdomen, caus-

The patient was transferred to the ICU and his condition sta-

ing compression of the stomach and shifting of the liver, spleen and

bilized. After 2 days, he was taken back to the OR for removal of

both kidneys posteriorly (Fig. 1). Inﬁltration of the diaphragm and

the packs and to ensure hemostasis. Upon opening the abdominal

the pericardium (Fig. 2) was observed, but there was no free ﬂuid.

wound and removing the packs, we observed a collected hematoma

The initial diagnosis was a gastrointestinal stromal tumor (GIST) or

over the liver bed and a second laceration bleeding from the upper

peritoneal mesothelioma because of its proximity and suspected

surface of the right hepatic lobe. This was managed and controlled

origin from the stomach. Further, the tumor was not suspected to

with Surgicel. Two drains were inserted and hemostasis was main-

have originated from the liver because of its large size and loca-

tained. The abdomen was closed in layers and the patient was

tion, the displacement of all the organs in the upper abdomen, and

transferred to the ICU in good condition. The patient was extu-

normal liver function test.

bated after 2 days and was stable hemodynamically for 6 days,

but on the following 7th day, he had hemodynamic ﬂuctuations

that required him to be re-intubated. An echocardiogram revealed

a bilateral atrial thrombus with left ventricular global hypokine-

sia. The patient coagulation proﬁle deteriorated with a drop in

hemoglobin level despite massive transfusions. The patient devel-

oped sepsis which led to acute renal shutdown. We found that

the source of sepsis was Acinetobacter which was detected in the

patient sputum and blood culture that could have caused the atrial

thrombus. All resuscitation measures were carried out, but the

patient showed no signs of improvement, continued bleeding from

all drains, and died shortly thereafter on day 16.

2.6. Final pathology report

The ﬁnal pathology report was a 30 cm 30 cm mixed

hepatoblastoma weighing 4 kg (Fig. 3) with positive immunohis-

tochemistry results for vimentin, focal alpha-fetoprotein and CEA

(Figs. 4).

3. Discussion

Hepatoblastoma is the most common primary malignant liver

neoplasm in children, and approximately 90% of all hepatoblas-

toma cases occur in patients under 5 years of age. Boys are affected

Fig. 2. The mass inﬁltrating the pericardium and compressing the liver, spleen and

left kidney. at twice the rate compared to girls. Hepatoblastoma in adolescent

CASE REPORT – OPEN ACCESS

206 B.O. Al-Jiffry / International Journal of Surgery Case Reports 4 (2013) 204–207

of hepatoblastoma. Hepatoblastoma is believed to arise from a

hepatic blastema, but this hypothesis seems to be inapplicable

to adult-onset hepatoblastoma. Patients more than 70 years old

have been diagnosed with hepatoblastoma, and the persistence of

primitive hepatic blastema for such a long period seems unlikely.

Furthermore, liver cirrhosis is not seen in childhood hepatoblas-

toma patients but is associated with 30% of adult hepatoblastoma

9–11

cases. This implies that these tumors may develop from differ-

ent pathogenic pathways in adults compared to children.

Tumors mostly occur as a single mass in the right or left liver

(unifocal), or in both livers (multifocal). Only one multifocal case

12,13

has been reported so far. The sizes of reported tumors range

from 2.5 to 25 cm. The tumor in our patient arose from the left

liver, was unifocal, and measured 30 cm × 30 cm.

The usual presentation of hepatoblastoma is loss of energy, loss

of weight, and a rapidly enlarging upper abdominal mass. Some

12,13

patients present with pain, fever and vomiting. In our patient,

the ﬁrst indication was upper abdominal pain, followed by a rapidly

enlarging mass.

The initial diagnosis of HB is mainly based on imaging. Proper

diagnosis, staging and treatment of HB require accurate imaging

studies. In this particular case the tumor was large, originated from

the periphery of the left liver, and displaced all the upper abdominal

organs, which made the diagnosis somewhat difﬁcult. Ultrasono-

Fig. 3. HB with Hx & E staining.

graphy is non-invasive and is particularly useful for examining

infants; the tumor is seen as a hyperechoic, solid, intrahepatic

and young adults is extremely rare, with only 45 cases reported in mass. Other standard methods include computed tomography,

literature, and the prognosis is much worse than that in childhood magnetic resonance imaging, and measurement of serum AFP and

␤

cases because they are usually diagnosed late. Studies on hepato- -HCG levels. However, ﬁnal diagnosis relies on tumor biopsy. Hep-

blastoma in adults have revealed a slight female preponderance. atoblastoma is extremely rare in adults, and the initial symptoms

The age of patients reported in the literature range from 17 to 82. are non-speciﬁc because of which it is often overlooked. Diagnosis

In our case, the patient was a 22-year-old man. of adult hepatoblastoma is also complicated by its similarities to

The etiology of hepatoblastoma has been elusive, but cytoge- other types of tumors such as hepatic teratoma, carcinosarcoma,

netic and molecular genetic abnormalities in hepatoblastoma have malignant mesenchymal tumor and hepatocellular carcinoma, and

revealed involvement of chromosomal loci on 1q, 2 (or 2q), 4q, 8 preoperative needle biopsy is contraindicated by nearly all authors

7 12

(or 8q), and 20. Loss of heterozygosity and imprinting at locus 11p if the surgeon is attempting surgical resection. One could argue

15.5 is also suggested to be a genetic basis for hepatoblastoma. that the use of transhepatic biopsy in this case, followed by neoad-

Accumulation of nuclear ␤-catenin implies oncogenic alteration juvant therapy could have helped in decreasing the tumor bulk.

of the WNT/␤-catenin pathway. Nuclear p53 accumulation indi- However, because of bleeding (vascular tumor as seen on CT), the

cates that a p53 mutation is also involved in the pathogenesis fear of rupture (large size) and our belief that the tumor could be

excised completely, in addition to other reasons mentioned above,

transhepatic biopsy was not done.

Thus, diagnosis of hepatoblastoma is not made until tumor

biopsy or autopsy. The usual pre-operative diagnosis is hepato-

cellular carcinoma, hepatic amoebic abscess or even abdominal

aortic aneurysm and in the present case the preoperative diag-

nosis was GIST. AFP and CEA were not detected and biopsy was not

done because of the fear of spread and rupture of the tumor; also,

with the diagnosis of GIST or sarcoma usually biopsy is contraindi-

cated. Hepatoblastoma could be suspected in middle aged patients

presenting with a primary liver lesion that is large in size and not

showing any risk factors for hepatocellular carcinoma like hepati-

tis. On the contrary, primary liver tumor is rarely seen in young

adults without hepatitis. In this case the tumor was very large and

we did not know its origin otherwise we would have suspected

hepatoblastoma, as we have mentioned above. This is the reason

why other diagnosis was made.

Complete surgical resection is the primary treatment for

patients with hepatoblastoma and is the only chance for opti-

mal clinical outcome. Despite this, improvements in survival

have occurred over the last three decades due to chemother-

apy regimens that reduce tumor size and enable complete tumor

15,16

excision even for initially unresectable or metastatic disease.

Chemotherapy not only shrinks tumors, but also makes them less

prone to bleeding and delineates them from the surrounding tis-

Fig. 4. HB with Alfa-fetoprotien-positive staining. sues to facilitate their resection. Hepatoblastoma is sensitive to

CASE REPORT – OPEN ACCESS

B.O. Al-Jiffry / International Journal of Surgery Case Reports 4 (2013) 204–207 207

chemotherapeutic agents such as doxorubicin, cisplatin, vin- and Dr Yasir Al-Nemary from Taif University for collecting the his-

cristine, 5-FU and cyclophosphamide. In the present case, the tory of the patient.

tumor was at an advanced stage, but was completely resected

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