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

Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervous system. The survival depends on the histology, the extension of the surgical removal, the efficacy of the complementary treatment (chemotherapy and cranio-spinal irradiation) in relation with the age of the children.We report our experience in Lyon concerning 151 patients treated from 1997 to 2021. All patients were recorded in the French Register of Pineal tumors which is centralized in Lyon since 2010.The analysis shows that benign tumors have a good survival rate and a total surgical removal is manadatory. Concerning true pineal tumors, pinealoblastomas have a bad prognostic especially in children aged inferior to three years old. The new pathological classification allows a better stratification with different groups identified and also the identification of a DICER 1 syndrome in family of patients with a pinealoblastoma that need medical investigations. Results of Germ Cell Tumors are more favorable with a global survival rate of 87 % and a rate of survival for pure germinomas of almost 97%. Results for gliomas of the pineal gland, that are tectal plate gliomas, are good for pilocytic astrocytomas while for other gliomas the prognosis is related to the grade of malignancy and the efficacy of complementary treatment. Papillary tumors need a complete removal for the best chance of survival. Finally, AT/TR still have a bad prognosis.Our results show that many progresses have to be accomplished for pineal region tumors and that these pathologies need a multidisciplinary approach to choose the best treatment to improve the survival rate and the patients’ quality of life.