What treatment approach do you take in a pediatric patient less than 3 years old with malignant papillary epithelioid tumor within the pineal region?

This is an interesting case for a rare tumor, especially in the pediatric population. There is unfortunately no prospective data so clinical management would be drawing from small retrospective series with primarily adult patients. The youngest patient with PTPR in the current literature is 5 years old. However, the most consistent finding among the adult cases reported is that gross total resection is associated with good PFS. Metastatic recurrence seems uncommon but has been reported - every published series reports 1-2 cases of metastatic recurrence but it is difficult to interpret this data as there was no standard treatment approach. In cases with a high mitotic index, focal RT may be an additional consideration to address the risk of ...