Posterior Fossa Tumours in the First Year of Life: A Two-Centre Retrospective Study.

Stefania Picariello,Lucia Quaglietta,Maria Elena Errico,Lucia De Martino,Alessandra Marini,Shlomi Costantini,Jonathan Roth,Fabio Savoia,Maria Serena De Santi,Giuseppe Cinalli,Giancarlo Nicosia,Giuseppe Mirone,Pietro Spennato,Nir Shimony

doi:10.3390/diagnostics12030635

Abstract

Posterior fossa tumours (PFTs) in infants are very rare, and information on these tumours is scarce in the literature. This retrospective study reports their pathological characteristics and describes surgical aspects and treatment outcomes. A two-centre cohort of infants with PFTs treated from 2007 to 2018 was retrospectively reviewed. Patient characteristics, clinical, and treatment data were reviewed. Survival curves for progression-free survival (PFS) and overall survival (OS) were generated. Thirty-three infants were retrieved. There were 11 low grade and 22 high-grade tumours. The most common presenting symptom was intracranial hypertension. Fifteen children out of thirty-three progressed. Five-year PFS was significantly lower in children with high-grade tumours (38.3%) than those with low-grade tumours (69.3%), p = 0.030. High-grade pathology was the only predictor of progression (HR 3.7, 95% CI 1.1–13.31), p = 0.045. Fourteen children with high-grade tumours died, with a 5-year OS of 55.25%. PFTs in children below one year of age still represent a unique challenge. Infants with high-grade tumours display the worst outcomes and the lowest survival, indicating that more effective strategies are needed.

Highlights

Primary CNS (Central Nervous System) tumours are the most common solid tumours in children, ranging between 16% and 23% of all paediatric malignancies [1–5]
From 2007 to 2018, 33 children under 12 months of age underwent a surgical procedure for a posterior fossa tumour in our Departments (18 at Santobono-Pausilipon Children’s Hospital, and 15 at Tel Aviv Medical Center)
We focus on the clinical presentation, the histopathological diagnosis, the surgical management of hydrocephalus, treatment, and survival outcome of 33 infants with posterior fossa tumours diagnosed and followed over a decade

Summary

Introduction

Primary CNS (Central Nervous System) tumours are the most common solid tumours in children, ranging between 16% and 23% of all paediatric malignancies [1–5]. In children older than one year, over two-thirds of intracranial tumours arise from the cerebellum or brainstem [6,7]. The infratentorial location is very rare in children below one year of age; relevant aspects of clinical, therapeutic, and biological characterisation are still undefined [8–11]. Signs of raised intracranial pressure, such as macrocephaly, bulging fontanel, and vomit, are the most common onset manifestations, though infants can present with more subtle clinical features, including hypotonia, irritability, poor feeding, failure to thrive, drowsiness, abnormal eye movement, lethargy, and a delay in developmental milestones [9,12]. Additional signs and symptoms might be found depending on the tumour location and involvement of anatomical structures and cranial nerves [13,14]

Objectives

Methods

Results

Discussion

Conclusion