Relapses of optic pathway tumors after first-line chemotherapy

Chemotherapy is accepted as first-line conservative treatment of optic pathway tumors in patients younger than 5. Limited data are available on the outcome of patients with recurrence/progression after initial chemotherapy.

Data on 68 children with Optic Pathway Tumors (OPT) treated with first-line Baby Brain (BBSFOP) chemotherapy at the Gustave Roussy Institute in Villejuif between 1990 and 2005 were reviewed.

During a median follow-up of 6 years, 44 (65%) patients were diagnosed with one or more relapses. Most of the relapses occurred during the first 6 years of life. Overall and progression-free survival rates at 5 years after first relapse were 64% and 14%, respectively. First relapse was treated with chemotherapy, radiotherapy or surgery in 28, 9, and 6 patients, respectively. Best response to second-line chemotherapy was partial response in 10, stable disease in 10, and progressive disease in 8 patients. Patients with objective radiologic response to first-line chemotherapy, had a greater chance to respond again to second-line chemotherapy (RR = 90% vs. 15%, P = 0.003). Median time to progression after first relapse was 1.7, 2.5, and 3.1 years after surgery, chemotherapy and radiotherapy, respectively. Finally, 25 (37%) patients received radiotherapy at a median age of 6.7 years.

Second-line chemotherapy can be effective in the treatment of relapses after first-line chemotherapy and delay further the need for RT, especially in patients whose tumor initially responded to chemotherapy. Despite the desire to avoid irradiation in treatment of young patients with OPT, radiotherapy was used for 37% of patients, usually before the age of 10.