Role of surgery, radiotherapy and chemotherapy in papillary tumors of the pineal region: a multicenter study

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CLINICAL STUDY

Role of surgery, radiotherapy and chemotherapy in papillary tumors of the pineal region: a multicenter study F. Fauchon • M. Hasselblatt • A. Jouvet • J. Champier • M. Popovic • R. Kirollos • T. Santarius • S. Amemiya • T. Kumabe • D. Frappaz • M. Lonjon • M. Fe`vre Montange • A. Vasiljevic

Received: 31 August 2012 / Accepted: 29 December 2012 / Published online: 12 January 2013 Ó Springer Science+Business Media New York 2013

Abstract Papillary tumor of the pineal region (PTPR), recently described as a distinct clinicopathological entity, can show aggressive biological behavior. The optimal therapeutic approach of PTPR has not been well defined. The role of surgery, radiotherapy, and chemotherapy in the treatment of PTPR was analyzed in a large multicenter series. In order to determine factors that influence prognosis, outcome data of a series of 44 patients with histopathologically proven PTPR were retrospectively analyzed. Of the 44 patients, 32 were still alive after a median follow-up of 63.1 months. Twelve patients experienced progressive disease, with seven undergoing two relapses and five more than two. Median overall survival (OS) was not achieved. Median progression-free survival (PFS) was 58.1 months. Only gross total resection F. Fauchon, M. Hasselblatt, M. Fe`vre Montange and A. Vasiljevic contributed equally to this study.

and younger age were associated with a longer OS, radiotherapy and chemotherapy having no significant impact. PFS was not influenced by gross total resection. Radiotherapy and chemotherapy had no significant effect. This retrospective series confirms the high risk of recurrence in PTPR and emphasizes the importance of gross total resection. However, our data provide no evidence for a role of adjuvant radiotherapy or chemotherapy in the treatment of PTPR. Keywords Papillary tumor of the pineal region  Pediatric  Prognosis  Radiosurgery Introduction Tumors located in the pineal gland region are rare neoplasms, constituting 0.5–1 % of all intracranial tumors [1],

F. Fauchon Centre de Haute Energie, Centre de Radiothe´rapie Prive´ and ADeRTU, Nice, France

R. Kirollos  T. Santarius Neurosurgery, Addenbrooke’s Hospital, University of Cambridge, Cambridge, UK

M. Hasselblatt Institute of Neuropathology, University Hospital Munster, Munster, Germany

S. Amemiya Department of Radiology, University of Tokyo, Tokyo, Japan

A. Jouvet  A. Vasiljevic Service de Pathologie et de Neuropathologie, Centre de Biologie et Pathologie Est, Groupement Hospitalier Est, Lyon, France A. Jouvet  J. Champier  M. Fe`vre Montange (&) Equipe Neuro-Oncologie et Neuro-Inflammation, Centre de Recherche en Neurosciences de Lyon, INSERM U1028, CNRS UMR5292, Universite´ de Lyon, 69372 Lyon, France e-mail: [email protected]

T. Kumabe Department of Neurosurgery, Tohoku University, Sendai, Japan D. Frappaz De´partement d’Oncologie Pe´diatrique, Centre Le´on Be´rard, Lyon, France M. Lonjon Service de Neurochirurgie, Hoˆpital Pasteur, Nice, France

M. Popovic Institute of Pathology, Facul