title: Management and outcome of chordomas in the pediatric population: The Hospital for Sick Children experience and review of the literature.
abstract: Chordomas are tumors arising from remnants of the embryological notochord, most commonly found in the spheno-occipital, spinal, or sacro-coccygeal areas. They are rare tumors in the pediatric population and are challenging to manage due to their difficult accessibility, proximity to important anatomy and extension into adjacent structures. We report a series of 10 children treated for chordoma at The Hospital for Sick Children focusing on their surgery, adjuvant therapy and long-term outcomes. A retrospective review involving patient charts, radiographic imaging, and pathology slides was performed for 10 chordoma patients during the period from 1987-2015. Important variables, including patient demographics, chordoma location, presentation, imaging characteristics, pathology subtype, treatment options, and long-term outcome were analysed. The series consists of seven girls and three boys with cranial or upper cervical spine chordomas. One patient presented with an extradural left cerebellopontine angle chordoma demonstrating aggressive and dedifferentiated features, which, to our knowledge, has not been previously described in the literature. All patients received surgical resection followed by photon or proton radiotherapy. Four patients with chondroid or atypical pathology also received chemotherapeutic adjuvants. All patients with classical pathology achieved favourable outcome, while the four patients with atypical pathology progressed quickly despite aggressive therapy, suggesting that pathology subtype is a crucial prognostic factor. This study summarizes 30years of surgical and adjuvant therapy experience in a large academic center for pediatric chordoma patients. Patient outcomes were dependent on pathology subtype, and a multidisciplinary approach involving surgery, radiotherapy, and chemotherapy can be considered on an individual basis.
authors: Department of Neurosurgery, Salford Royal Foundation Trust Manchester, United Kingdom; Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
authors: Vassilios
authors: V
authors: Tsitouras
authors: Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Department of Biostatistics and Epidemiology, Harvard T. H. Chan School of Public Health, Boston, MA, USA.
authors: Shelly
authors: S
authors: Wang
authors: Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Division of Neurosurgery, Department of Surgery, Hospital for Sick Children, Suite 1503, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
authors: Peter
authors: P
authors: Dirks
authors: Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Division of Neurosurgery, Department of Surgery, Hospital for Sick Children, Suite 1503, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
authors: James
authors: J
authors: Drake
authors: Division of Hematology and Oncology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Eric
authors: E
authors: Bouffet
authors: Department of Pathology, University of Toronto, Hospital for Sick Children Toronto, Ontario, Canada.
authors: Cynthia
authors: C
authors: Hawkins
authors: Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Suzanne
authors: S
authors: Laughlin
authors: Division of Neurosurgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada; Division of Neurosurgery, Department of Surgery, Hospital for Sick Children, Suite 1503, 555 University Avenue, Toronto, ON M5G 1X8, Canada. Electronic address: James.rutka@sickkids.ca.
authors: James T
authors: JT
authors: Rutka
mesh_terms: D000293:Adolescent; D002574:Cervical Vertebrae / Q000601:surgery; D059186:Chemoradiotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D002817:Chordoma / Q000000981:diagnostic imaging / Q000601:surgery / Q000628:therapy*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures; D012189:Retrospective Studies; D019292:Skull Base Neoplasms / Q000000981:diagnostic imaging / Q000601:surgery / Q000628:therapy; D013125:Spinal Neoplasms / Q000000981:diagnostic imaging / Q000601:surgery / Q000628:therapy*; D016896:Treatment Outcome
keywords: Chemotherapy; Chordoma; Neurosurgery; Pediatric; Radiation; Skull base