Neuroblastoma in adolescents

Background: Less than 5% of neuroblastomas are diagnosed in adolescent patients. Previous studies of patients who were treated with less intensive chemotherapy regimens relative to currently available regimens suggested that adolescents survived longer than younger children, and this finding was related to a lack of myc-N amplification. Those reports prompted the authors to study a cohort of adolescent patients who had been included in more recent trials.

Methods: The authors investigated the presentation, treatment, and outcome in 28 adolescent patients who were enrolled in studies of the French Society of Pediatric Oncology during the period from 1987 to 1999 and who were older than age 10 years at the time they were diagnosed with neuroblastoma. The results were used to compare this subpopulation with a control group of children.

Results: None of the six patients with Stage I-II disease either developed recurrent disease or died. At 5 years, disease progression was high (progression-free survival [PFS], 28%) for the 9 adolescents with Stage III disease, but so was survival (overall survival [OS], 86%). The 13 adolescent patients with metastatic neuroblastoma had very poor outcomes (PFS, 18%; OS, 27%). Despite intensive therapy, advanced neuroblastoma appeared to carry a poorer prognosis in adolescent patients compared with children, although patients with Stage III disease had a more indolent course. No difference was found between adolescent patients and children regarding the clinical presentation, treatment schedule, or doses and tolerance of chemotherapy. The incidence of elevated urinary catecholamine metabolite secretion was lower in adolescents compared with children.

Conclusions: Adolescent patients with advanced neuroblastoma had less favorable outcomes compared with children, even if survival in adolescents with Stage III disease seemed longer.