Endocrine tumor syndromes and their genetics

This book on the genetics of endocrine tumours has been authored by leading experts in the field. It is the 41^st volume of the ongoing series, ‘Frontiers of Hormone Research’. The Chapter by J. A. Carney, ‘Carney Triad’ is a good example of how clinicians recognize unusual tumour syndromes and identify phenotypes which serve as the starting point of research into tumourigenesis. The chapters on common endocrine tumours such as familial non-medullary thyroid cancer and endocrine tumours in other familial neoplasia are interesting and welcome additions, and expand the spectrum of genetic disorders that predispose to endocrine tumours.

The chapters are well organized and coherent. Each chapter highlights the syndrome under consideration, a brief historical background, the constellation of clinical features and the underlying genetic abnormality. Wherever it is sorted out, the intracellular events that lead to tumourigenesis have been outlined. Wherever established, the genotype-phenotype correlation is presented clearly and where an individual phenotype has multiple predisposing genotypes the issue has been clarified. Each chapter has tables that present a summary of the chapter under consideration. The images are appropriate and of good quality. There are simplified diagrams which lead to a better understanding of cellular events in tumourigenesis. Each chapter outlines the indications for genetic testing, the current recommendations for screening relatives of the index case and the current recommendations regarding treatment. The rationale behind choosing therapeutic targets based on an understanding of the underlying pathophysiology and new potential therapeutic targets have been outlined.

To summarize, this book is a must read for physicians, paediatricians, endocrinologists and basic scientists.