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The spontaneous rupture of hepatoblastoma in childhood is a rare life-threatening event. Despite never being firmly documented this event is considered to play a negative impact on long term survival. Herewith the clinical history of a 6-month-old child with a very large mass and intratumoral bleeding at the diagnosis, who suffered of spontaneous tumor rupture after two cycles of pre-operative chemotherapy and ultimately died of tumor recurrence despite appropriate treatment, is reported. In addition to this case the pertinent literature regarding the prognostic implication of tumor rupture in childhood hepatoblastoma (at the time of diagnosis or during treatment) is reviewed. This report is meant to focus the problem of the clinical and prognostic relevance of tumor rupture and to stimulate appropriate large scale studies aimed to investigate the prognostic value of this event, and to identify the clinical tumor characteristics which may predict its occurrence.

We report the use of high dose chemotherapy with peripheral blood stem cell rescue as a consolidation treatment for a 3-year-old child affected by metastatic hepatoblastoma, who achieved complete lung response only after conventional treatment. The patient is presently alive 27 months after high dose chemotherapy with blood stem cell rescue with no evidence of disease. The role of high dose chemotherapy with blood stem cell rescue to consolidate the complete clearing of lung disease in metastatic hepatoblastoma remains controversial; the data available in the literature and our experience seems to suggest to keep this treatment option open to further consideration in the clinical setting of high-risk patients.