Subtotal Resection with Proton-Beam Radiotherapy for Treatment of Pineal Parenchymal Tumor of Intermediate Differentiation in a Pediatric Patient

Pineal parenchymal tumors of intermediate differentiation (PPTIDs) are rare, pinealocyte-derived brain tumors that occur primarily in adults. The clinical prognosis fits somewhere between the benign pineocytoma and highly malignant pineoblastoma. There is very little published literature regarding this tumor in children and the existing pediatric information is enfolded with adult data in single-center reviews. The most common treatment recommendation for adults is aggressive resection, possibly followed by craniospinal irradiation (CSI) and/or chemotherapy. However, the adult literature is inconsistent, often contradictory, and does not address specific considerations in pediatric patients. To our knowledge, there are no papers specifically addressing the management and clinical considerations of PPTID in pediatric patients. As such, the optimal treatment strategy in children is unknown.

We describe the treatment of a 6-year-old child who presented with obstructive hydrocephalus from a PPTID. The child was treated with a partial tumor resection followed by localized proton beam radiation. He has been followed for 8 years. Clinically, he is doing well, and his most recent MRI shows negligible residual tumor with no sign of recurrence.

Our case suggests safe resection followed by proton beam radiotherapy may be effective in treating children with this exceedingly rare entity. While further study is needed, this strategy may avoid unnecessary surgical risk and the consequences of CSI on the developing pediatric nervous system.