2 year old male child presents with lump in epigastric region since 1 month. USG abdomen was done

Ultrasound images:

USG shows well defined mixed echogenic lesion seen predominantly in left lobe of liver showing vascularaty with dilated vascular channel within. The patient was further evaluated.

Discussion
Hepatoblastoma is the most common liver cancer in children, although it is relatively uncommon compared with other solid tumors in the pediatric age group.. Complete surgical resection of the tumor at diagnosis, followed by adjuvant chemotherapy, is associated with 100% survival rates, but the outlook remains poor in children with residual disease after initial resection, even if they receive aggressive adjuvant therapy.

Pathophysiology
Hepatoblastomas originate from immature liver precursor cells and present morphologic features that mimic normal liver development. Hepatoblastomas are usually unifocal and affect the right lobe of the liver more often than the left lobe. Micro vascular spread can extend beyond the apparently encapsulated tumor.

Grossly, the tumor is a tan bulging mass with a pseudocapsule. Cirrhosis is not associated with this tumor. Metastases affect the lungs and the porta hepatis; bone metastases are very rare. CNS involvement has been reported at diagnosis and during relapse.
Loss of heterozygosity (LOH) of chromosome arm 11p markers occurs commonly in hepatoblastoma identified in association with Beckwith-Wiedemann syndrome (BWS) and hemihypertrophy. Isochromosome 8q is seen in mixed hepatoblastomas
Patients with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas. Germline mutations in the APC tumor suppressor gene occur in patients with FAP, and mutations in the APC tumor suppressor gene are frequently detected in the colonic polyps and adenocarcinomas associated with FAP.

Frequency
Hepatoblastoma accounts for 79% of all liver tumors in children and almost two thirds of primary malignant liver tumors in the pediatric age group. Approximately 100 cases of hepatoblastoma are reported per year. A significantly higher rate of hepatoblastomas is observed among low birth weight (LBW) and very low birth weight (VLBW) infants born prematurely.

Sex
Males are typically affected more frequently than females; the male-to-female ratio is 1.7:1.

Age : Hepatoblastoma usually affects children younger than 3 years, and the median age at diagnosis is 1 year. Hepatoblastoma is very rarely diagnosed in adolescence and is exceedingly rare in adults. Occasionally, nests of hepatoblastoma cells are found in hepatocellular carcinoma lesions; this is more common in adults than in children.

Abdominal radiography
Plain abdominal films reveal a right upper quadrant abdominal mass. Calcification is seen in approximately 6% of hepatic masses and 12% of hemangiomas.

Ultrasonography
Abdominal ultrasonography allows assessment of tumor size and anatomy, which helps in surgical planning.The mass usually appears hyperechoic on abdominal ultrasound images, which is particularly useful in determining vascular involvement (vessels have lower attenuation than surrounding parenchyma).

CT scanning
CT scanning of the abdomen using contrast reveals patchy enhancement. CT scanning reveals involvement of nearby structures. Regional lymph nodes are almost never involved.
CT scanning of the chest is warranted to assess for pulmonary metastases.

MRI: This is believed to be superior to CT scanning but does not necessarily add to the anatomic detail seen on CT scans.
Radionuclide bone scanning: This is recommended to evaluate for bone metastases when a patient is symptomatic.

References:

• Ang JP, Heath JA, Donath S, Khurana S, Auldist A. Treatment outcomes for hepatoblastoma: an institution's experience over two decades. Pediatr Surg Int. Feb 2007;23(2):103-9.

• Otte JB, Pritchard J, Aronson DC, et al. Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience. Pediatr Blood Cancer. Jan 2004;42(1):74-83.

• Otte JB, de Ville de Goyet J, Reding R. Liver transplantation for hepatoblastoma: indications and contraindications in the modern era. Pediatr Transplant. Oct 2005;9(5):557-65.

Contributed by:
Dr. Vijay jadhav (junior resident)
Dr. Meenakshi agarwal (junior resident)
Dr. Vidya Jadhav (junior resident)
Dr. Abhishek andhare ( junior resident)
Dr. Unmesh bhosale (junior resident)

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