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This book provides a detailed body of information important to an understanding of the clinical aspects and management of such a challenging disease. The authors strive to provide the physician with insights and illustrations as well as useful tips and tricks for the everyday clinical practice. Several world-known experts in the field participated in this fruitful effort offering their knowledge and everyday experience, as well as valuable and original photos from their personal archives as well as the most recent and up-to-date guidelines in the field. In this book, we evaluate all the challenging pathways from the moment of diagnosis to the surgical and medical therapies. The pathogenesis, the variation of the morphological types and the problems in differential diagnosis are also discussed. 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