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Book Review: Chordomas: Technologies, Techniques, and Treatment Strategies

Sen, Chandranath MD

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doi: 10.1093/neuros/nyx394
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This book is intended to be a comprehensive book about chordomas. The authors have done a nice job pulling together all the current information to give the reader an overview of chordoma and its treatments. It is organized into 4 sections and the chapters in each section are arranged logically. Although most of the chapters are written by the editors, there are multiple authors involved. The chapters are well written and there is an up to date list of references at the end of each chapter.

The first section deals with some general topics, beginning with some historical facts and epidemiology. The survival data over the years are presented, highlighting that this is still a potentially lethal disease in most cases. Chapter 3 describes the evolution of the notochord and its key role in the normal development of the embryo and, interestingly, the origin of this tumor, which in turn is controlled by the brachyury gene. Over the last several years, there has been considerable research unraveling the genetics and molecular structure of the tumor. The present state of the knowledge is described in the fourth and fifth chapters. Metastasis and local seeding at the time of surgery occurs in a limited number of patients and is described in Chapter 6. Chapter 7 discusses the light and electron microscopic features of the chordoma. Until recently, the molecular biology of chordomas was poorly understood. In the last few years, some investigators have successfully created chordoma cell lines in Vitro and also in animal models and have been validated to represent the original tumor. These models are now being used to study the action of various drugs. The current state of this effort is described in Chapter 8.

The next section describes the magnetic resonance imaging (MRI), computed tomography (CT), and molecular imaging of skull base and spinal chordomas. Chordomas have a classical and distinct appearance on MRI and CT scans, but a list of differential diagnoses needs to be entertained when viewing these imaging studies. Chapters 9, 10, and 11 are dedicated to imaging of the skull base and spinal chordomas. The authors describe the features distinguishing a chordoma from other tumors. There are various radionuclide markers that are also used to detect chordomas, especially recurrent and metastatic chordomas.

The third and the largest section deals with the surgical treatment, and the majority of this deals with skull base chordomas. Although skull base chordomas arise in the clivus, they often extend into a variety of neighboring areas and compartments, both intra- and extradural. No single approach will be adequate to access and remove all clival chordomas. Chapters 14 to 20 deal with surgical decision making and the variety of surgical approaches available for the treatment of skull base chordomas. Each approach is described along with its indications and limitations.


Instability of the craniovertebral junction often results from removal of tumors in this area. Chapter 21 describes the various ways of reconstructing and stabilizing this junction. All of the chordomas involving the entire mobile spine down to the sacrum are condensed in Chapter 22. Adjuncts to surgery, such as image guidance, intraoperative neurophysiology, as well as intraoperative imaging that improve the degree and safety of resection are described in Chapter 23.

Radiation therapy, which is an integral part of treatment of chordomas, has become very sophisticated from the initially disappointing results from fractionated radiotherapy. The final section has 3 chapters describing the various modern radiation techniques and their results, usually after aggressive surgical resection. Almost all the discussion concerning radiation is described in context of skull base chordomas. In Chapter 24, Dr Al-Mefty provides some pearls from his personal observations over the many years of his practice treating skull base chordomas. His thoughts are similar to the other contemporary surgeons who treat chordomas.

Chordomas have a high tendency to recur and a variably metastasize. Recurrent chordomas are very difficult to manage, and Chapter 28 discusses the options available once the tumor recurs. At the present state of the art, the only options are repeat surgery and sometimes radiation. No effective chemotherapy exists at this time.

Treatment of chordomas in any part of the body can have a substantial negative impact on the patient's quality of life. Chapter 29 discussed these negative effects on a patient's life and function. Again, this chapter is mainly concerned with skull base chordomas. Spinal chordomas are just as important to be considered in this context but there is not much discussed here.

Chordomas in the pediatric population are even less frequent than in adults. It is a special problem as described in Chapter 30. The authors have included chondrosarcomas in this discussion, which I do not find necessary. Although it looks similar in the imaging studies, this is a vastly different tumor from chordomas and the main thing for the reader is not to confuse the 2 entities.

After reading the book thus far, the reader will realize that despite the advances in the surgery and radiation techniques in treating chordomas, the recurrence rate and survival results leave a lot to be improved on. With the advances in the molecular studies, the next frontier in treatment will be identification of drugs targeting specific markers to be used to control incompletely resected or recurrent tumors. The final Chapter 31 provides some direction but not much is discussed with respect to the current state of drug therapy and results. This is an area of significant investigation at this time and should have merited a separate chapter from oncologists working on this.

Overall, this is a useful reference for surgeons, medical oncologists, radiation oncologists, and basic scientists studying chordomas and treating patients. Since this is such a rare disease, this book will appeal to a limited audience. The reference list after each chapter is comprehensive. The only shortcoming I would mention is that the book is heavily weighted towards skull base chordomas.


The author has no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.

Copyright © 2017 by the Congress of Neurological Surgeons