Chordomas: Technologies, Techniques, and Treatment Strategies

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Review by L Flood
Middlesborough, UK

It is very much to the credit of any publisher to produce something of this quality, on what is a highly specialised topic. The end result is surely the last word on...’ for some years to come, even if the audience is limited. Contributors include neurosurgeons (obviously the majority input), head and neck surgeons, radiologists and pathologists, and some very clever basic scientists. 

My recollection of this rare tumour is of a slowly growing, relatively silent thing, which will get you in the end, whatever you do. The opening paragraph encouraged me, telling me that the advances described here have doubled the survival rate over the last 30 years. Alas, chapter two tells us that median survival is still only 4.7 years. We all have hazy memories that this originates from the notochord, and possibly presents to us when it arises in the clivus or craniocervical junction. My limited experience was of an operation, performed on a jelly-like substance, a procedure that owed more to a sucker than to a scalpel or biopsy forceps. Harvey Cushing, the first surgeon to remove a cranial chordoma, lamented from a surgical standpoint complete extirpation of such a growth is unthinkable. Palliative measures alone must be resorted to. Imaging, microsurgery, endoscopy, radiotherapy and stereotactic radiosurgery have transformed that. Immunotherapy seems an exciting prospect here. Indeed, there is excellent coverage of molecular biology, and I am now familiar with brachyury, even if I do find it hard to say. That chapter alone has 151 references! Once I then moved onto the cytogenetics chapter, it did dawn on me just how scientific and updated this book proved to be. 

Fear not. We are soon in familiar territory, with computed tomography and magnetic resonance imaging of skull base lesions not easily missed. As always with Thieme, the imaging is printed to a very high quality. Chapters follow on surgical decision making and approaches. The anterior approach to the clivus and especially endoscopy is relevant to us, but the neurosurgeons will persist in finding their own routes of access.

A very thoughtful, indeed a brilliant, chapter is entitled Chordomas. A Personal Perspective. Subsections are headed Only Chordoma is Chordoma(i.e. it is not chondrosarcoma, a point also stressed in James Rutkas chapter), Chordoma is not a Benign Disease(I will honestly admit that I did not know they could metastasise) and Once it Recurs, It Never Leaves(when treatment becomes palliative). Closing chapters cover irradiation, particle beam therapy and stereotactic radiosurgery.

This book will appeal more to neurosurgeons, but, fortunately for us, it does concentrate far more on the skull base than the lower end of the spine. Rhinologists in training, or those already proficient in getting into the sphenoid and then carrying on, will find this invaluable.

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