Overview
Hepatoblastoma is a rare malignant liver tumor predominantly affecting children, though it can occur in adults with extremely poor prognosis. Advances in multimodal treatment strategies have improved outcomes, particularly in pediatric cases 123.Diagnosis
Preoperative diagnosis often relies on imaging studies (ultrasound, CT, MRI) and biopsy 17.
Elevated alpha-fetoprotein (AFP) levels are common and useful for monitoring 12.
Cytogenetic analysis may reveal specific abnormalities, such as pseudodiploid karyotypes 8.Management
First-line treatment: Multimodal approach including intensive preoperative chemotherapy (e.g., cisplatin, THP-ADR) tailored to age 2.
Surgery: Curative resection of the primary tumor when feasible 12.
Adjuvant therapy: Short-course adjuvant chemotherapy post-surgery to prevent recurrence 1.
Radiological interventions: Trans-arterial embolizations for managing complications like bleeding 2.
Targeted therapy: Additional chemotherapy for metastatic disease 1.Special Populations
Pediatrics: Improved prognosis with multimodal therapy, especially in infants 23.
Familial cases: Higher incidence in families with familial adenomatous polyposis (FAP) 4.
Genetic syndromes: Presence in patients with trisomy 18 5.
Siblings/cousins: Rare familial occurrences noted, suggesting potential genetic predisposition 46.Key Recommendations
Employ multimodal treatment including intensive preoperative chemotherapy tailored to age for optimal tumor reduction before surgery (Evidence: Moderate 2).
Consider curative resection when feasible following chemotherapy, followed by adjuvant chemotherapy to enhance survival rates (Evidence: Moderate 1).
Utilize radiological interventions such as trans-arterial embolizations for managing complications like bleeding (Evidence: Weak 2).
Monitor alpha-fetoprotein levels for diagnosis and treatment response (Evidence: Expert opinion 12).References
1 Nakamura S, Sho M, Kanehiro H, Tanaka T, Kichikawa K, Nakajima Y. Adult hepatoblastoma successfully treated with multimodal treatment. Langenbeck's archives of surgery 2010. link
2 Ueno S, Hirakawa H, Yokoyama S, Hinoki T, Tobita K, Ohtani Y et al.. Treatment of infantile hepatoblastoma and related complications. The Tokai journal of experimental and clinical medicine 2005. link
3 Finegold MJ. Tumors of the liver. Seminars in liver disease 1994. link
4 Bernstein IT, Bülow S, Mauritzen K. Hepatoblastoma in two cousins in a family with adenomatous polyposis. Report of two cases. Diseases of the colon and rectum 1992. link
5 Mamlok V, Nichols M, Lockhart L, Mamlok R. Trisomy 18 and hepatoblastoma. American journal of medical genetics 1989. link
6 Surendran N, Radhakrishna K, Chellam VG. Hepatoblastoma in siblings. Journal of pediatric surgery 1989. link80110-1)
7 Daniel E, Kifle A. An unusual presentation of hepatoblastoma. Ethiopian medical journal 1989. link
8 Petković I, Nakić M, Cepulić M. Cytogenetic analysis of hepatoblastoma. Cancer genetics and cytogenetics 1985. link90180-3)
9 Akoojee SB, Coovadia YM. Hepatoblastoma. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1978. link