Case of the Week #540

Glynis Sacks*, Cerine Jeanty**

Affiliation:
*Vanderbilt University, Nashville, Tennessee, USA
** St Mary's Medical Center, San Francisco, California, USA

Posting Dates: July 1, 2021 - July 14, 2021

Case Report: 35-year-old G2P1 woman with no past medical or family history presented with a normal ultrasound at 20 weeks of gestation. She had no pregnancy complications and at 37 weeks, 5 days, the following findings were noted:

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Video 1

These images are obtained at follow up 2 weeks later.

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Answer

Our prenatal imaging was suggestive of a hepatoblastoma.

Ultrasound demonstrated an enlarging, relatively homogeneous hypoechoic mass in the right upper quadrant with well-defined borders. No internal vascularity was detected with Doppler interrogation.

The baby was born at term and postnatal ultrasound and MRI showed an unresectable tumor. The infant therefore underwent liver transplant at 4 months of age. He is now 2 years old, though does have multiple medical problems.

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Image 9: Transverse image of the liver
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Image 10: Transverse image of the liver
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Image 11: Transverse image through the IVC
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Discussion

While hepatoblastoma is rare, occurring in 1.6 cases per million [1], it is the most common liver tumor in children under 2 years of age [2]. The etiology is unknown, though aberrations in the Wnt signaling pathway have been implicated [3]. The tumor is assumed to originate in utero as the incidence is highest at birth and histologically, hepatoblastoma cells resemble embryonal liver cells [4]. Congenital anomalies can be present in 5.5% of patients [2] and hepatoblastoma has been associated with very low birth weight (<1500g), Beckwith–Wiedemann syndrome, and familial adenomatous polyposis [3]. Obstetrical complications may include premature birth, polyhydramnios, and hydrops [5].

Several cases of hepatoblastoma have been prenatally diagnosed in the literature [5,6,7]. Prenatal diagnosis not only allows for adequate patient counselling, but also coordination with pediatric surgeons, neonatologists, and oncologists at a tertiary care center. The diagnosis is typically made in the third trimester with the visualization of a well-demarcated, solid spherical mass, usually in the right lobe of the liver. This is hypothesized to be related to the lower oxygen tension on the right side supplied by the portal vein, compared to the left side, which is supplied by oxygenated blood from the umbilical vein [6]. The mass may have a “spoke-wheel” appearance on ultrasound. With color Doppler, hepatoblastoma often has a vascular rim, as well as vascularity within the tumor itself. Tumor peak systolic flow of 40 cm/s or more has been associated with a malignant hepatic tumor compared to hemangioma [8].

The differential diagnosis includes hepatocellular carcinoma, mesenchymal hamartoma, hemangioendothelioma, metastatic lesions such as neuroblastoma, leukemia and lymphomatous infiltration [6,7]. Mesenchymal hamartoma, hemangioendothelioma, and vascular tumors are often cystic. On doppler, hepatocellular carcinoma often shows tortuous vessels in the stroma arising from the adjacent parenchyma, but the vascular rim at the periphery is not typical. Metastatic lesions on doppler show a vascular rim, however the stroma is relatively avascular [6].

Previously, reported prenatal cases have had poor outcomes [6,7, 9]. We report a prenatally diagnosed case of hepatoblastoma that went on to receive liver transplant and is alive at 2 years of age. Overall, 5-year survival in patients with hepatoblastoma is 82% [1]. Patients with fetal-predominant histology have better survival outcomes [2]. Approximately two-thirds of patients present with unresectable disease however, hepatoblastoma is chemo-sensitive and these patients receive neo-adjuvant chemotherapy. This renders patients surgically resectable in 75-85% of cases [2,4]. Survival is 70% in patients who receive neoadjuvant chemotherapy, surgical resection and subsequent adjuvant chemotherapy [10]. If they have major vascular involvement or multifocal disease, they may be liver transplantation candidates. Survival in patients who receive liver transplant is anywhere between 65-80% [2,10].

References
[1]    Howlader N, Noone AM, Krapcho M et al. SEER Cancer Statistics Review, 1975-2018, National Cancer Institute. Bethesda, MD, https://seer.cancer.gov/csr/1975_2018.
[2]    Kasahara M, Ueda M, Haga H, et al. Living-donor liver transplantation for hepatoblastoma. Am J Transplant. 2005 Sep;5(9):2229-35.
[3]    Lim IIP, Bondoc AJ, Geller JI, Tiao GM. Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation. Children (Basel). 2019 Jan; 6(1): 1.
[4]    Khaderi S, Guiteau J, Cotton RT, et al. Role of liver transplantation in the management of hepatoblastoma in the pediatric population. World J Transplant. 2014 Dec 24;4(4):294-8.
[5]    Wang JY, Zheng QZ, Cao DY et al. Prenatal Diagnosis of Congenital Hepatoblastoma. Maternal-Fetal Medicine. 2020 Apr;2(2):115-118.
[6]    Shih JC, Tsao PN, Huang SF, et al. Antenatal diagnosis of congenital hepatoblastoma in utero. Ultrasound Obstet Gynecol. 2000 Jul;16(1):94-7.
[7]    Ergin H, Yildirim B, Dagdeviren E, et al. A prenatally detected case of congenital hepatoblastoma. Pathol Oncol Res. 2008 Mar;14(1):97-100.
[8]    Numata K, Tanaka K, Mitsui K, et al. Flow characteristics of hepatic tumors at color Doppler sonography: correlation with arteriographic findings. AJR Am J Roentgenol 1993;160(3):515–521.
[9]    Ammann RA, Plaschkes J, Leibundgut K (1999) Congenital hepatoblastoma: a distinct entity. Med Pediatr Oncol 32:466–468.
[10] Ismail H, Broniszczak D, Kaliciński P, et al. Changing treatment and outcome of children with hepatoblastoma: analysis of a single center experience over the last 20 years. J Pediatr Surg 2012; 47: 1331-1339.

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