How would you manage a recurrent pineoblastoma following prior CSI?

Recurrent pineoblastoma following prior CSI represents a challenging clinical scenario. As there is very little published data to guide optimal management, our approach is adopted from recurrent medulloblastoma. The prognosis for patients with recurrent medulloblastoma/PNET is poor, with 5-year OS <10% in previously irradiated children.¹ Among relapsing patients on the HIT-SIOP-PNET4 trial, treatment consisted of combinations of surgery (25% of relapsed patients), focal radiotherapy (22%), high dose chemotherapy with stem cell rescue (21%) and conventional chemotherapy (90%). Choosing the optimal treatment is complex and depends on several factors including presence of symptoms, unifocal vs. multifocal recurrence, response to previous thera...