title: Successful treatment of primary intracranial sarcoma with the ICE chemotherapy regimen and focal radiation in children.
abstract: OBJECT Primary CNS sarcomas are very rare pediatric tumors with no defined standard of care. METHODS This study was a retrospective review of children diagnosed with a primary CNS sarcoma and treated at 2 Canadian tertiary care centers between 1995 and 2012. This report focuses on patients with cerebral hemispheric tumor location due to their specific clinical presentation. RESULTS Fourteen patients with nonmetastatic primary CNS sarcoma were identified; in 9 patients, tumors were located in the cerebral hemisphere and 7 of these patients presented with intratumoral hemorrhage. One infant who died of progressive disease postoperatively before receiving any adjuvant therapy was not included in this study. The final cohort therefore included 8 patients (4 males). Median patient age at diagnosis was 11.8 years (range 5.8-17 years). All tumors were located in the right hemisphere. Duration of symptoms prior to diagnosis was very short with a median of 2 days (range 3-7 days), except for 1 patient. Three (37.5%) patients had an underlying diagnosis of neurofibromatosis Type 1 (NF1). Gross-total resection was achieved in 5 patients. The dose of focal radiation therapy (RT) ranged between 54 Gy and 60 Gy. Concomitant etoposide was administered during RT. ICE (ifosfamide, carboplatin, etoposide) chemotherapy was administered prior to and after RT for a total of 6-8 cycles. Seven of the 8 patients were alive at a median time of 4.9 years (range 1.9-17.9 years) after treatment. CONCLUSIONS In this retrospective series, patients with primary CNS sarcomas located in the cerebral hemisphere most commonly presented with symptomatic acute intratumoral hemorrhage. Patients with NF1 were overrepresented. The combination of adjuvant ICE chemotherapy and focal RT provided encouraging outcomes.
authors.affiliation: Division of Pediatric Hematology, Oncology, and Bone Marrow Transplantation, and.
authors.forename: Lucie
authors.initials: L
authors.lastname: Lafay-Cousin
authors.affiliation: Division of Paediatrics.
authors.forename: Gillian
authors.initials: G
authors.lastname: Lindzon
authors.affiliation: Division of Neurosurgery.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Taylor
authors.affiliation: Division of Pediatric Neurosurgery, Alberta Children's Hospital, Calgary, Alberta;
authors.forename: Walter
authors.initials: W
authors.lastname: Hader
authors.affiliation: Division of Neuropathology, and.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Hawkins
authors.affiliation: Division of Radiation Oncology, Tom Baker Cancer Center, Calgary, Alberta; and.
authors.forename: Robert
authors.initials: R
authors.lastname: Nordal
authors.affiliation: Department of Radiation Oncology, Princess Margaret Hospital, Toronto, Ontario, Canada.
authors.forename: Normand
authors.initials: N
authors.lastname: Laperriere
authors.affiliation: Division of Neuro-Radiology, The Hospital for Sick Children, Toronto, Ontario;
authors.forename: Suzanne
authors.initials: S
authors.lastname: Laughlin
authors.affiliation: Division of Haematology/Oncology, Paediatric Brain Tumour Program.
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.affiliation: Division of Haematology/Oncology, Paediatric Brain Tumour Program.
authors.forename: Ute
authors.initials: U
authors.lastname: Bartels
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000150:complications / Q000188:drug therapy* / Q000532:radiotherapy; D002170:Canada; D016190:Carboplatin / Q000008:administration & dosage; D054022:Cerebrum; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D020300:Intracranial Hemorrhages / Q000150:complications; D008297:Male; D017253:Neurofibromatoses / Q000150:complications; D012189:Retrospective Studies; D012509:Sarcoma / Q000150:complications / Q000188:drug therapy* / Q000532:radiotherapy; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D005047:Etoposide; D016190:Carboplatin; D007069:Ifosfamide
keywords: CNS sarcoma; GTR = gross total resection; ICE = ifosfamide, carboplatin, etoposide; MCA = middle cerebral artery; MINST = malignant intracerebral nerve sheath tumor; NF1 = neurofibromatosis Type 1; OS = overall survival; PFS = progression-free survival; RT = radiation therapy; STR = subtotal resection; adjuvant chemotherapy; brain tumor; intracranial hemorrhage; oncology