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Medulloblastoma of the adult: results from a multicenter retrospective study by AINO (Italian Association of Neuro-Oncology) and SIN (Italian Society of Neurology)

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Abstract

Introduction

Medulloblastoma (MB) is the most common primary malignant intracranial tumor in childhood, but it is very rare in adults, and for this reason, the optimal treatment has not yet been defined. We designed a multicentric study in order to define relevant outcome measures for future prospective studies.

Materials and methods

The project involved 10 Italian centers. The database shared among the centers contains epidemiological, diagnostic (radiological and histological/molecular), therapeutic, recurrence information, and survival data.

Results

A total of 152 patients (102 males and 50 females, median age 32) were included in the study. Twenty-three of 152 patients had a diagnosis of classic medulloblastoma, 52/152 had desmoplastic/extensive nodularity, 2/152 had large-cell anaplastic medulloblastoma, and the remaining had diagnoses not otherwise specified. Almost all patients underwent craniospinal irradiation after surgery; in 85.5% of patients, adjuvant chemotherapy, mainly platinum- and etoposide-based chemotherapy, was performed immediately after RT. Upon recurrence, most patients were retreated with various chemotherapy regimens, including intrathecal chemotherapy in patients with leptomeningeal dissemination. The overall survival (OS) rate at 5 years was 73.3% (95% CI, 65.0–80.0%). The median OS for the whole group of patients was 112 months.

Conclusions

The data collected were mainly consistent with the literature. A limitation of this study was the large number of patients lost to follow-up and the lack of molecular data for most patients diagnosed until 2010. An important challenge for the future will be MB biologic characterization in adults, with the identification of specific genetic patterns. It will be important to have more national and international collaborations to provide evidence-based management strategies that attempt to obtain a standard of care.

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Authors and Affiliations

  1. Neuro-oncology Unit, Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy

    Paola Gaviani, Giorgia Simonetti & Antonio Silvani

  2. Department of Neuro-Oncology, University of Turin and City of Health and Science University Hospital, Turin, Italy

    Roberta Rudà & Federica Franchino

  3. Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy

    Giuseppe Lombardi

  4. Radiotherapy Department, Businco Oncological Hospital, Cagliari, Italy

    Marco Possanzini & Sara Squintu

  5. Neuro-Oncology Unit, Regina Elena Cancer Institute, Rome, Italy

    Veronica Villani

  6. Department of Experimental and Clinical Biomedical Sciences, Radiation Oncology Unit, University of Florence, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy

    Mariaausilia Teriaca

  7. Neurology Unit, Neuromotor & Rehabilitation Department, Azienda USL-IRCCS of Reggio Emilia, Reggio Emilia, Italy

    Francesco Cavallieri

  8. Department of Neurosurgery, University of Messina, Messina, Italy

    Maria Caffo

  9. Neurological Department, ASST, Lecco, Italy

    Andrea Salmaggi

  10. Neurosurgery “Maggiore della Carità” University Hospital, Corso Mazzini 18, 28100, Novara, Italy

    Andrea Bianco

  11. Neurology VIII-Molecular Neuro-Oncology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy

    Elena Anghileri

  12. Unit of Neuroepidemiology, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milan, Italy

    Mariangela Farinotti

  13. Department of Research and Clinical Development, Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy

    Irene Tramacere

Authors
  1. Paola Gaviani
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  2. Giorgia Simonetti
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  3. Roberta Rudà
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  4. Federica Franchino
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  5. Giuseppe Lombardi
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  6. Marco Possanzini
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  7. Sara Squintu
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  8. Veronica Villani
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  9. Mariaausilia Teriaca
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  10. Francesco Cavallieri
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  11. Maria Caffo
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  12. Andrea Salmaggi
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  13. Andrea Bianco
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  14. Elena Anghileri
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  15. Mariangela Farinotti
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  16. Irene Tramacere
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  17. Antonio Silvani
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Corresponding author

Correspondence to Giorgia Simonetti.

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The authors declare that they have no conflict of interest.

Ethical approval

This study has been approved by the Ethics Committee of the IRCCS Istituto Neurologico Carlo Besta.

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Gaviani, P., Simonetti, G., Rudà, R. et al. Medulloblastoma of the adult: results from a multicenter retrospective study by AINO (Italian Association of Neuro-Oncology) and SIN (Italian Society of Neurology). Neurol Sci 42, 665–671 (2021). https://doi.org/10.1007/s10072-020-04556-6

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  • DOI: https://doi.org/10.1007/s10072-020-04556-6

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