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Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome
Authors:A. Sandri  N. Sardi  L. Genitori  F. Giordano  P. Peretta  M. E. Basso  D. Bertin  L. Mastrodicasa  L. Todisco  F. Mussa  M. Forni  U. Ricardi  L. Cordero di Montezemolo  E. Madon
Affiliation:(1) Pediatric Oncology Department, University of Turin, Torino, Piazza Polonia, 94, 10124 Turin, Italy;(2) Division of Pediatric Neurosurgery, “Meyer” Hospital, Firenze, Italy;(3) Neurosurgery Department, “Regina Margherita Children’s Hospital,”, Torino, Italy;(4) Department of Pathology, “Regina Margherita Children’s Hospital,”, Torino, Italy;(5) Department of Radiotherapy, University of Turin, Torino, Italy;(6) Pediatric Hematology Department, University of Turin, Torino, Italy
Abstract:Objective Brainstem tumors (BSTs) are usually gliomas and are divided into diffuse BSTs (DBSTs) and focal BSTs (FBSTs). The aim of this study is to investigate the different outcomes of these two entities.Methods Thirty-one patients with BSTs were admitted to our institution from 1995 to 2003. Patients with DBSTs were treated with locoregional radiotherapy (1.8 Gy/day for 54 Gy) and weekly vincristine for radiosensitization (1.5 mg/sm for six total doses). Patients with FBSTs underwent surgical resection. Chemotherapy and/or radiotherapy were considered in progression.Results and conclusions Fourteen patients were diagnosed as having DBSTs. The responses to treatment were ten cases of partial response, three of stable disease, and one of progressive disease. General and/or neurological symptoms improved in more than 80% of patients. The median time from diagnosis to progression and to death were, nonetheless, 8 (range of 3–13) and 13 (range of 4–25) months, respectively, with a 2-year overall survival rate of 12.3% [standard error (SE) 11.2]. Seventeen patients were diagnosed as having FBSTs. Gross total removal was achieved in 4/17 cases, subtotal removal in 7/17, and partial removal in 6/17. There was one surgery-related death. Eight out of 17 patients had adjuvant chemo- and/or radiotherapy after progression: 6/8 are without neurological symptoms and 2/8 have died due to tumor progression. The 4-year overall and disease-free survival rates are 87.4 (SE 8.4) and 58.8% (SE 11.9), respectively, the extent of resection being the most important prognostic factor (p=0.012). DBSTs continue to carry a dismal prognosis, thus demanding new treatment modalities; FBSTs can be treated surgically and patients benefit from a better prognosis.
Keywords:Brainstem tumors  Children  Posterior fossa surgery  Chemotherapy  Radiotherapy
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