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 nervous system. The survival depends on the histology, the extension of the surgical removal, the e cacy 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 classi cation allows a better strati cation with different groups identi ed and also the identi cation 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 e cacy 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.Out 891 tumors 316 patients (33%) are children: 165 of the 316 patients (54%) have been treated in different French centers and 151(46%) have been treated in Lyon and are considered for this report.Results of the French register were already published in a previous paper. [6] Materials And Methods All pediatric patients that have been treated from January 1997 to December 2020 in Lyon in the Department of Pediatric Neurosurgery have been considered. All these patients are recorded in the French Register of Pineal Tumors. 151 patients were eligible for the study with an age between one years to eighteen years old. The sex ratio was of 1.2 males /Girl.The symptomatology was characterized in 54% by signs of intracranial hypertension with headaches and vomiting. Ophthalmological troubles were present in 21% of patients, endocrinological problems were associated in 5% of cases, while in others 18% of patients the clinical manifestations were characterized by more insidious manifestations as asthenia, tiredness and gradual lowering of scholar performances.All patients were studied with a cerebro-spinal MRI with and without Gadolinium with angio-MRI sequences to study arterial feeders and the venous structures. The CT-scan was realized in some patients performed in peripheral hospitals. We never realized angiography by the Seldinger technique for the surgical p...

Treatment of Pineal Region Lesions in Children

“…However, pineal region lesions often have extensive tissue heterogeneity and mixed cell populations 14 . Most types of region lesions have a poor response to radiotherapy 15 . Moreover, we demonstrated that many pineal region lesions could not be completely removed with radiotherapy, even when the lesions were diagnosed as germinomas by neuroimaging, tumor markers, or plasma hormones.…”

“…14 Most types of region lesions have a poor response to radiotherapy. 15 Moreover, we demonstrated that many pineal region lesions could not be completely removed with radiotherapy, even when the lesions were diagnosed as germinomas by neuroimaging, tumor markers, or plasma hormones. We believe that mixed tumors may be the reason for this phenomenon.…”

“…Hydrocephalus is the most common complication of pineal lesions, occurring in 54% to 91% of children with hydrocephalus secondary to pineal region lesions 3,15 . CSF diversion before resection is performed routinely in some centers to manage hydrocephalus; 29 however, the necessity of CSF diversion before resection for children with pineal region lesions remains controversial.…”

“…Of the 54 children, 46 were diagnosed with hydrocephalus secondary to pineal region lesions. A total of 24 patients underwent a pineal lesion resection and 15…”

“…8,28 CSF Diversion Before Resection is Necessary for Children with Pineal Region Lesions Hydrocephalus is the most common complication of pineal lesions, occurring in 54% to 91% of children with hydrocephalus secondary to pineal region lesions. 3,15 CSF diversion before resection is performed routinely in some centers to manage hydrocephalus; 29 however, the necessity of CSF diversion before resection for children with pineal region lesions remains controversial. Some investigators believe that most children with hydrocephalus secondary to pineal lesions do not need diversion.…”