While a number of international meetings over the past two decades have indeed focused on adrenal tumors in the context of hormone excess (Cushing’s syndrome, hyperaldosteronism, and pheochromocytoma), few have exclusively catered to the science and clinical care of ACC and those afflicted with the disease. With only a single FDA-approved drug for ACC (mitotane – a derivative of the pesticide DDT), institutional experiences varied widely until recently when historic biases have slowly yielded to data-driven treatment strategies. A large part of the impetus for this push has come from Europe, where the availability of country-wide integrated networks for treatment has allowed a small number of centers in Italy, France, and Germany (among others) to develop specific expertise and specific treatment protocols for this rare disease.
In an attempt to facilitate coordination of global efforts, a consensus conference was organized and held at the University of Michigan in September 2003. At that meeting, an international group of physicians and scientists with research interest and clinical expertise in ACC set up initial guidelines for the diagnosis and treatment of ACC. Three principles emerged. Successful treatment of ACC demands coordinated care in the context of a multidisciplinary team dedicated to the disease. Future therapies for ACC need to be predicated on hypothesis-driven research based on a thorough analysis of tumor biology. Lastly, major advancement in the field demands national and international collaborative networks to facilitate analysis of large datasets and coordinate future clinical trials. The FIRM-ACT (First International Randomized Trial in locally advanced and Metastatic Adrenocortical Cancer Treatment) that coordinated over 35 ACC centers in a single multinational trial set precedence for the actualization of these principles. The Second International Adrenal Cancer Symposium: Clinical and Basic Science held at the University of Michigan in March 2008 built upon the momentum of the 2003 consensus meeting and the successful development of a large international ACC network through the FIRM-ACT trial.
As opposed to a solitary review article, a textbook with multiple chapters dedicated to discrete topics in the field provides contributors the opportunity to objectively review historic data and detail the current state of clinical care and research accomplishments. While this is a major advantage of a textbook, it is also a major challenge for a book that focuses solely on a rare cancer where data are scant. Each individual chapter covers well established knowledge in the area, but also allows room for expert opinion. Lastly, because ACC has been linked to several genetic disorders that usually escape discussion in a focused review of adrenal tumors, the various syndromes will be discussed in their entirety in separate chapters. The 32 Chapters of the 9 Sections are authored by the scientific and clinical leaders in the field. The overall goal of this book is therefore to provide definitive reference material for scientists and clinicians, to introduce trainees to concepts of ACC management, and to stimulate further research, future collaborations, and networking.
Part I History of Adrenocortical Carcinoma Research and Clinical Care
- 1 The History of Adrenocortical Carcinoma Treatment – A Medical Perspective
- 2 The History of Adrenocortical Carcinoma Treatment – A Surgical Perspective
- 3 Epidemiology of Adrenocortical Carcinoma
- 4 Clinical Presentation and Initial Diagnosis
- 5 Diagnostic Approach to Incidentaloma
- 6 Computed Tomography/Magnetic Resonance Imaging of Adrenocortical Carcinoma
- 7 Functional Imaging of Adrenocortical Carcinoma
- 8 Classical Histopathology and Immunohistochemistry
- 9 Cellular and Molecular Pathology of Adrenocortical Carcinoma
- 10 Overview of Genetic Syndromes Associated with Adrenocortical Cancer
- 11 Li–Fraumeni Syndrome
- 12 TP53 Molecular Genetics
- 13 Telomeres and Telomerase in Adrenocortical Carcinoma
- 14 Beckwith–Wiedemann Syndrome
- 15 The Insulin-Like Growth Factor System in Adrenocortical Growth Control and Carcinogenesis
- 16 WNT/ß-Catenin Signaling in Adrenocortical Carcinoma
- 17 Adrenocortical Stem and Progenitor Cells: Implications for Cancer
- 18 Adrenocortical Cell Lines
- 19 Mouse Models of Adrenal Tumorigenesis
- 20 Overview of Treatment Options for Adrenocortical Carcinoma
- 21 Chemotherapy
- 22 Mitotane
- 23 Pharmacotherapy for Hormone Excess in Adrenocortical Carcinoma
- 24 Surgery for Adrenocortical Carcinoma
- 25 Radiation Therapy for Adrenocortical Carcinoma
- 26 Follow-Up and Monitoring of Adrenocortical Carcinoma
- 27 Aldosterone-Producing Adrenocortical Carcinoma
- 28 Adrenocortical Cancer in Children
- 29 Genome-Wide Studies in Adrenocortical Neoplasia
- 30 New Strategies for the Treatment of Adrenocortical Carcinoma
- 31 The Dutch Adrenal Network
- 32 The ENS@T Initiative
Product Details
- Hardcover: 546 pages
- Publisher: Springer; 1st edition (November 24, 2010)
- Language: English
- ISBN-10: 0387772359
- ISBN-13: 978-0387772356
- Product Dimensions: 9.2 x 6.2 x 1.5 inches