Pineoblastomas : A long-term follow up study of three cases in Helsinki Neurosurgery

Abstract

Background Pineoblastomas are very rare malignant lesions with a bad prognosis and high mortality during the first five years from diagnosis. Report of cases We present a retrospective review of three patients with histologically confirmed pineoblastomas consecutively operated on between 1997 and 2015. One of our patients died >14 years after surgery, and the other 2 patients are still alive and in good condition without recurrence of the disease >12 years after surgery. All of them underwent gross total resection and craniospinal radiotherapy. Individualized scheme of chemotherapy was administered in two cases. The cornerstones for the surgical resection of pineoblastomas are reported. Conclusions A proper multidisciplinary management of pineoblastomas, which associates gross total microsurgical resection of the lesion and an adjuvant therapy determined by our neurooncology team based on accurate craniospinal adjuvant radiotherapy with boost of radiation on the tumoral bed, and when needed, an adequate but aggressive medulloblastoma-like chemotherapy, may improve the overall survival of these malignant lesions.