Hepatic non-Hodgkin lymphoma and hepatoblastoma complicating ataxia-telangiectasia

Patients with ataxia-telangiectasia (A-T) have an increased risk of developing malignancies and are prone to severe early or late toxicity owing to chemotherapy. Leukemia and lymphoma account for about 85% of malignancies, but solid tumors have also been reported. We describe an unusual case of an 8-year-old child affected by A-T, who presented a primary hepatic B-cell non-Hodgkin lymphoma, treated with reduced doses of R-CHOP cycles plus rituximab. Three years later, the patient developed hepatoblastoma as a second malignancy. This case clearly emphasizes the need for intensive monitoring of A-T patients for early signs of malignancy and the opportunity to consider specific and modified regimens of chemotherapy.