This book provides a comprehensive review of the epidemiology, pathogenesis, diagnosis, and management of chordomas of the mobile spine and sacrum. Historically, knowledge about how to deliver such state-of-the-art care has not been widespread, resulting in inconsistent treatment, and, all too often, suboptimal outcomes for chordoma patients. This text is an important step towards broadening that knowledge, and, in turn, improving the care provided to chordoma patients. The book is divided into 4 parts comprising 16 chapters. The first part focuses on the pathophysiology and molecular drivers of chordoma. The second focuses on the epidemiology and clinical history, as well as the histological, oncologic, and radiographic work-up of chordoma. The third part focuses primarily on the technical aspects of surgery for chordoma. It is broken down by anatomic region, with the final two chapters focusing on the soft tissue and bony reconstruction following chordoma resection. The last part focuses on the exciting field of adjuvant therapies for chordoma. This includes both radiation therapies and novel chemotherapeutic options for recurrent, metastatic, and dedifferentiated chordoma.

Written by world experts from leading chordoma centers, Chordoma of the Spine serves as a valuable reference for spinal oncologists and general spine surgeons who may encounter chordoma patients in their practice. It provides the most up-to-date evidence regarding all aspects of treating patients with this disease. By focusing on this specific pathology of the spine, this resource condenses expert-level research into understandable treatment algorithms.

Daniel M. Sciubba
Department of Neurosurgery
Zucker School of Medicine at Hofstra University/Northwell Health
Manhasset, NY, USA

Joseph H. Schwab
Department of Orthopaedic Surgery
Massachusetts General Hospital
Harvard Medical School



Section I Pathophysiology and Molecular Mechanisms of Chordoma
1 Notochordal Morphogenesis and the Origin of Chordoma Matthew L. Goodwin, MD PhD, David C. Clever, MD PhD 2 Molecular and Genetic Mechanisms of Spinal ChordomaConnor Liu, BS; Ann Liu, MD; Chetan Bettegowda, MD/PhD
Section II Epidemiology, Clinical History, and Work-Up for Chordoma
3 Historical Overview, Demographics, and Clinical Presentation of Spinal Chordoma Zach Pennington BS, Andrew Schilling AB, Joseph H. Schwab MS MS, Daniel M. Sciubba MD 4 Clinical Work up, Diagnostic Imaging and Biopsy Daniel Ryan, MD; John Gross, MD; Zach Pennington BS, Majid Khan, MD 5 Histopathologic Classification of Spinal Chordoma John M Gross MD, Edward F McCarthy MD 6 Multi-disciplinary Planning for Treatment Nicholas A Shepard MD, Matthew W Colman MD
Section III Surgical Management of Chordoma
7 Surgical Management of Chordoma of the Occipitocervical Junction Zach Pennington BS, Jeff Ehresman BS, Andrew Schilling BA, Daniel M. Sciubba MD 8 Surgical Management of Chordoma of the Cervical Spine Oliver G.S. Ayling MD, MSc, Nicolas Dea MD, MSc 9 Surgical Management of Chordoma of the Thoracic Spine Daniel G. Tobert MD, John H. Shin MD, Joseph H. Schwab MD 10 Surgical Management of Chordoma of the Lumbar Spine Sutipat Pairojboriboon MD, Amanda Sacino MDPhD, Sheng-fu Larry Lo MD 11 Surgical Management of Chordoma of the Sacrum Peter S. Rose, MD, Matthew T. Houdek, MD, Cory G. Couch, MD 12 Soft Tissue Reconstruction Following Surgery for Sacral Chordoma Rachel Skladman MD, Zach Pennington BS, Justin M. Sacks MD MBA FACS 13 Vascular Reconstruction After EnBloc Resection. Daniel G. Tobert MD, Joseph H. Schwab MD
Section IV Radiation and Chemotherapeutic Adjuvants for Chordoma
14 Photon Therapy for Chordoma of the Spine Chunzi Jenny Jin MD MSc, Yoshiya Josh Yamada MD FRCPC 15 Heavy Particle Therapy for Chordoma David J. Konieczkowski MD PhD, Reiko Imai MD, Thomas F. DeLaney MD 16 Systemic Therapy, Trials and Future Directions for Chordoma of the Spine Daniel J. Zabransky, MD, PhD, Zach Pennington BS, Christian Meyer, MD, MS, PhD
Index