Long-term outcome of posterior fossa medulloblastoma in patients surviving more than 20 years following primary treatment in childhood

Radek Frič,Bård Kronen Krossnes,Tryggve Lundar,Arild Egge,Petter Brandal,Einar Stensvold,Paulina Due-Tønnessen,Bernt Johan Due-Tønnessen

doi:10.1038/s41598-020-66328-8

Radek Frič, Bård Kronen Krossnes + Show 6 more

Open Access

https://doi.org/10.1038/s41598-020-66328-8

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Journal: Scientific Reports	Publication Date: Jun 10, 2020
Citations: 15	License type: open-access

Affiliation: Oslo University Hospital, University of Oslo

Abstract

The aim of the study was to analyze the long-term outcome (>20 years) after treatment of posterior fossa medulloblastoma (MB) in childhood. We analyzed data from patients treated for posterior fossa MB between 1974 (introduction of the first international treatment protocol in Norway) and 1987 (when use of radiotherapy was abandoned in children under 4 years of age). Out of 47 children, 24 survived >20 years. At the time of analysis, 16 patients (median age 41 years, range 32–52) were alive (median follow-up 34 years, range 30–42), while 8 patients died 22–41 years (median 31 years) after primary treatment: one late death (after 22 years) was due to tumor recurrence whilst other 7 deaths (after 23 to 41 years) were related to the detrimental effects of the treatment (secondary tumors, stroke, severe epilepsy and depression). Observed 20- and 30-year survival rates were 51% and 44%, respectively. Despite successful treatment of MB in childhood and satisfactory tumor control during the first 20 years following primary treatment, our data indicates that even long-term survivors may die from tumor recurrence. However, the main factors causing late mortality and morbidity in long-term survivors seem to be the complications related to radiotherapy given in childhood.

Highlights

The aim of the study was to analyze the long-term outcome (>20 years) after treatment of posterior fossa medulloblastoma (MB) in childhood
The present analysis focuses on the subgroup of patients with posterior fossa MB who were treated at our institution from 1974 and to the end of 1987, and who survived the first 20 years following primary treatment
Out of 47 children primarily treated for their posterior fossa MB between 1974 and 1987, 21 died within the first five years, including one perioperative death, one death four days after surgery, and one patient who died after six months due to acute leukoencephalopathy following radiotherapy

Summary

Introduction

The aim of the study was to analyze the long-term outcome (>20 years) after treatment of posterior fossa medulloblastoma (MB) in childhood. While advances in the neurosurgical treatment can be documented by reduced perioperative and early postoperative mortality and morbidity1, 5- and 10-year survival rates for different neoplastic entities are considered a standard measure of outcome and quality of treatment for the neoplastic disease itself[2,3], along with increasing focus on survivors‘ quality of life[4,5,6] and physical functioning[7] It was well recognized already in the 1950’s and -60’s that there were different prognoses for children with various types of posterior fossa tumors[8]. As these ultra-late aspects have not been sufficiently reflected in the literature[13], we wish to summarize the experience from this particular subgroup of patients in the present study

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