Paola Gaviani1, Giorgia Simonetti2, Roberta Rudà3, Federica Franchino3, Giuseppe Lombardi4, Marco Possanzini5, Sara Squintu5, Veronica Villani6, Mariaausilia Teriaca7, Francesco Cavallieri8, Maria Caffo9, Andrea Salmaggi10, Andrea Bianco11, Elena Anghileri12, Mariangela Farinotti13, Irene Tramacere14, Antonio Silvani1. 1. Neuro-oncology Unit, Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy. 2. Neuro-oncology Unit, Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy. giorgia.simonetti@istituto-besta.it. 3. Department of Neuro-Oncology, University of Turin and City of Health and Science University Hospital, Turin, Italy. 4. Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Padua, Italy. 5. Radiotherapy Department, Businco Oncological Hospital, Cagliari, Italy. 6. Neuro-Oncology Unit, Regina Elena Cancer Institute, Rome, Italy. 7. Department of Experimental and Clinical Biomedical Sciences, Radiation Oncology Unit, University of Florence, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy. 8. Neurology Unit, Neuromotor & Rehabilitation Department, Azienda USL-IRCCS of Reggio Emilia, Reggio Emilia, Italy. 9. Department of Neurosurgery, University of Messina, Messina, Italy. 10. Neurological Department, ASST, Lecco, Italy. 11. Neurosurgery "Maggiore della Carità" University Hospital, Corso Mazzini 18, 28100, Novara, Italy. 12. Neurology VIII-Molecular Neuro-Oncology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy. 13. Unit of Neuroepidemiology, Fondazione IRCCS Istituto Neurologico "Carlo Besta", Milan, Italy. 14. Department of Research and Clinical Development, Scientific Directorate, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria, 11, 20133, Milan, Italy.
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.
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.
Authors: Benjamin H Kann; Nataniel H Lester-Coll; Henry S Park; Debra N Yeboa; Jacqueline R Kelly; Joachim M Baehring; Kevin P Becker; James B Yu; Ranjit S Bindra; Kenneth B Roberts Journal: Neuro Oncol Date: 2017-02-01 Impact factor: 12.300
Authors: Paul A Northcott; Andrey Korshunov; Vijay Ramaswamy; Hallie Coltin; Lakshmikirupa Sundaresan; Kyle S Smith; Patryk Skowron; Luca Massimi; Charles G Eberhart; Karisa C Schreck; Nalin Gupta; William A Weiss; Daniela Tirapelli; Carlos Carlotti; Kay K W Li; Marina Ryzhova; Andrey Golanov; Olga Zheludkova; Oksana Absalyamova; Konstantin Okonechnikov; Damian Stichel; Andreas von Deimling; Caterina Giannini; Scott Raskin; Erwin G Van Meir; Jennifer A Chan; Daniel Fults; Lola B Chambless; Seung-Ki Kim; Alexandre Vasiljevic; Cecile Faure-Conter; Rajeev Vibhakar; Shin Jung; Sarah Leary; Jaume Mora; Roger E McLendon; Ian F Pollack; Peter Hauser; Wieslawa A Grajkowska; Joshua B Rubin; Marie-Lise C van Veelen; Pim J French; Johan M Kros; Linda M Liau; Stefan M Pfister; Marcel Kool; Noriyuki Kijima; Michael D Taylor; Roger J Packer Journal: Acta Neuropathol Date: 2021-08-18 Impact factor: 15.887
Authors: Nazanin K Majd; Maximilan Mastall; Heather Lin; Seyede Shiva Dibaj; Kenneth R Hess; Ying Yuan; Manuela Martin-Bejarano Garcia; Gregory N Fuller; Kristin D Alfaro; Maria K Gule-Monroe; Jason T Huse; Soumen Khatua; Ganesh Rao; David I Sandberg; Jeffrey S Wefel; Debra N Yeboa; Arnold C Paulino; Susan L McGovern; Wafik Zaky; Anita Mahajan; Dima Suki; Shiao-Pei Weathers; Rebecca A Harriso; John F De Groo; Vinay K Puduvalli; Marta Penas-Prado Journal: Neurooncol Adv Date: 2021-06-22