Recurrent metastatic neuroblastoma followed by myelodysplastic syndrome: possible leukemogenic role of temozolomide

An 8-year old child had a pelvic MYCN-nonamplified neuroblastoma (NB) with retroperitoneal nodal extension. Multi-modality therapy achieved complete remission (CR). Small recurrences confined to left supraclavicular nodes were treated with surgery alone at 4.9, 6.5, 7.5, 9.5, and 12.9 years from diagnosis. Monitoring through 12 months after the last resection showed CR. When she returned 34 months later (16.8 years from diagnosis), she had massive disease in the left neck and upper trunk, without osteomedullary metastases. Salvage therapy featured 11 cycles of temozolomide. She developed myelodysplastic syndrome with 45,XX,der(7)t(7;21) (p15;q11),-21 at age 24 and refused treatment; 19 months later she was transfusion-dependent but her NB remained in CR.