Results of the treatment of pineal tumors in children: the Lyon experience.

Abstract

Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervoussystem. Patient survival depends on the histology, the extension of the surgical removal, and the efficacy of thecomplementary treatment (chemotherapy and cranio-spinal irradiation), as well as the age of the patient. In this study, we analyzed 151 pediatric patients treated for pineal tumors from 1997 to2020 in Lyon, France. All patients were recorded in the French Register of Pineal tumors, which has beencentralized and maintained in Lyon since 2010. Our analysis shows that benign tumors have an overall positive prognosis with total surgical removal.Concerning pineal parenchymal tumors, pinealoblastomas have a poor prognosis , especially in children less thanthree years old. A new pathological classification system allows for a better stratification of patient risk withindifferent groups of patients with pineal tumors. It is also important to note that the identification of DICER 1syndrome in families with pinealoblastomas warrant further medical investigation. Patients with Germ Cell Tumors have more favorable outcomes, with a global survival rate of 87 % and a puregerminoma survival rate of almost 97%. When analyzing the prognosis of pineal gland gliomas, otherwise knownas tectal plate gliomas, pilocytic astrocytomas had a promising prognosis. Otherwise, prognosis of other tectal plategliomas are related to the grade of malignancy and the efficacy of complementary treatment. Lastly, papillarytumors need a complete removal for the best chance of survival, and Atypical teratoid/ rabdoid tumors (AT/TR)still have a bad prognosis, regardless of surgical resection. Our results show that, with regards to pediatric pineal region tumors, there are still areas inprognostic indicators that need to be improved. Similarly, these pathologies need to be treated via amultidisciplinary approach to improve a patient's survival rate and their quality of life.