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. 2022 Mar 4;12(3):635.
doi: 10.3390/diagnostics12030635.

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

Stefania Picariello  1   2 , Pietro Spennato  3 , Jonathan Roth  4 , Nir Shimony  4 , Alessandra Marini  5 , Lucia De Martino  1 , Giancarlo Nicosia  6 , Giuseppe Mirone  3 , Maria Serena De Santi  3 , Fabio Savoia  7 , Maria Elena Errico  8 , Lucia Quaglietta  1 , Shlomi Costantini  4 , Giuseppe Cinalli  3
Affiliations

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

Stefania Picariello et al. Diagnostics (Basel). .

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.

Keywords: Atypical Teratoid/Rhabdoid Tumour; astrocytoma; ependymoma; infants; infratentorial tumours; medulloblastoma; posterior fossa tumours.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Whole-population Progression-Free Survival (PFS) and Overall Survival (OS) curves.
Figure 2
Figure 2
Progression-Free Survival curves by low-grade and high-grade pathology.
Figure 3
Figure 3
Overall survival curves by low grade and high-grade pathology.
Figure 4
Figure 4
A coronal T2W MRI (A) and axial contrast-enhanced T1 W MRI (B) showing a posterior fossa tumour involving the left cerebellopontine angle and extending into the fourth ventricle in a 5-month old infant. (C,D) Three-month follow-up MRI showing complete surgical resection and VP shunt. (E). An axial T2W MRI at 1-year follow-up showing recurrence (white circle) of the tumour in the left lateral recess. (F). Gross total resection of the tumour recurrence. (G). Metastatic dissemination 22 months after diagnosis (single lumbar metastasis). (H). Complete removal of the spinal lesion.
Figure 5
Figure 5
(A). A fourth ventricular tumour arising from the cerebellar vermis. (B). Complete Resection of a desmoplastic medulloblastoma. (C). Mycotic ventriculitis (D). Intraventricular haemorrhage.
See this image and copyright information in PMC

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References

    1. Stiller C.A., Bayne A.M., Chakrabarty A., Kenny T., Chumas P. Incidence of childhood CNS tumours in Britain and variation in rates by definition of malignant behaviour: Population-based study. BMC Cancer. 2019;19:139. doi: 10.1186/s12885-019-5344-7. - DOI - PMC - PubMed
    1. Williams T., Naushahi M., Bernard A., Campbell R. Epidemiology of paediatric central nervous system tumours in Queensland, Australia. J. Clin. Neurosci. 2021;92:126–130. doi: 10.1016/j.jocn.2021.07.054. - DOI - PubMed
    1. Ostrom Q.T., Cioffi G., Waite K., Kruchko C., Barnholtz-Sloan J.S. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014–2018. Neuro-Oncology. 2021;23:iii1–iii105. doi: 10.1093/neuonc/noab200. - DOI - PMC - PubMed
    1. Lannering B., Sandström P.-E., Holm S., Lundgren J., Pfeifer S., Samuelsson U., Strömberg B., Gustafsson G., Swedish Childhood CNS Tumor Working Group (VCTB) Classification, incidence and survival analyses of children with CNS tumours diagnosed in Sweden 1984-2005. Acta Paediatr. 2009;98:1620–1627. doi: 10.1111/j.1651-2227.2009.01417.x. - DOI - PubMed
    1. Arora R.S., Alston R.D., Eden T.O.B., Estlin E.J., Moran A., Birch J.M. Age-incidence patterns of primary CNS tumors in children, adolescents, and adults in England. Neuro-Oncology. 2009;11:403–413. doi: 10.1215/15228517-2008-097. - DOI - PMC - PubMed

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