Dissemination of knowledge is the best and only strategy capable of improving clinical management in rare and ultra-rare malignancies, including primary adrenal malignancies. It is preliminary to any further healthcare policy such as, for example, the centralization of cases in referral institutions.

Adrenal primary malignancies are probably the rarest among rare pathologies. This epidemiological consideration is at the origin of a vicious circle: the lack of preoperative diagnosis leads to inappropriate surgical procedures and to inadequate follow-up and systemic therapy strategies. Furthermore, when malignancy is recognized, the relative role of systemic, surgical, or other local ablative techniques is often misinterpreted. All this produces a negative impact on survival, also in patients who could have been cured with a correct therapeutic strategy.

This open access book provides a user-friendly exploration of these rare conditions, using a transversal approach common in modern precision medicine. The text covers various clinical aspects, including epidemiology, genetics, molecular medicine, imaging, and surgery. Guidelines for managing syndromic situations are provided. The book thoroughly addresses all the different surgical issues and emphasizes the importance of pathology in assessing prognosis. The pivotal role of medical oncologists in managing these patients is explored, along with alternative treatments such as nuclear medicine and radiotherapy. Finally, the book highlights the latest preclinical research and its implications for translational medicine.