TITLE:
Hepatoblastoma in Developing Countries; Eight Years of Single Centre Experience
AUTHORS:
Sayed Abdelahamid, Reham Abdelaziz Khedr, Madeeha El Wakeel, Alaa Younes, Gehad Ahmed, Naglaa Elkinaai, Marwa Tantawy, Hanafy Ahmed Hafez
KEYWORDS:
Hepatoblastoma, Risk Factors, Survival
JOURNAL NAME:
Journal of Cancer Therapy,
Vol.9 No.10,
October
9,
2018
ABSTRACT: Background and
objectives: Although hepatoblastoma (HB) is a rare childhood tumor and
constitutes only 0.9% of all pediatric cancers, there was an obvious
improvement in risk stratification and prognosis over the last two decades.
This study aimed to assess the outcome of HB patients treated in our center and
to investigate the impact of different prognostic factors on the survival of
these patients. Patients and methods: This was a retrospective study included newly diagnosed patients with HB
presented to the Children Cancer Hospital Egypt (CCHE 57357), from July 2007 to
June 2015. Patient’s data were analyzed for the clinical characteristics and
survival outcome of the included patients. Results: One hundred
twenty-four children were included during the study period with a median age of
14 months. The tumor was found occupying the entire liver in 25 patients (20%); while it was confined to one lobe in 80% of them,
portal vein thrombosis was diagnosed in 10 patients, and there were 30 patients
(24%) had metastatic disease at presentation. Only five patients (4%) underwent
primary surgical excision, and all of them were grossly excised (stage I); 77/119 (64.7%) experienced delayed surgery after
two to six courses (median, four courses) of C5VD and the overall resection
rate was 66%. There were 42 patients (35.3%) failed to do surgical excision
either because they still had evident metastatic disease with poor chemotherapy
response, or because their tumor remained unresectable after six courses of
chemotherapy. The 3-year event-free and overall survivals for the whole studied
patients were 45.7% (95% CI, 36.9% - 56.7%), and 66.7% (95% CI, 57.1% - 77.8%) respectively. The 3-year EFS and OS were significantly
better in those who underwent surgical excision (68.63% and 80.74%
respectively, P-value 0.001). Also, the survival rates were significantly
affected by the presence of metastatic disease at presentation, tumor stage and
initial risk grouping of the studied patients. Conclusion: Surgical
excision, tumor stage and COG risk grouping are the main prognostic variables
affecting patients’ outcome. Efforts to achieve resectability of the tumor
either by advanced surgical techniques or by developing effective preoperative
treatment, especially for advanced and metastatic disease, are mandatory.