AIMN, ITANET, SIE joint position paper for the recognition, prevention and management of NET carcinoid syndrome and crises during peptide receptor radionuclide therapy

Purpose: Carcinoid syndrome (CS) is the most frequent neuroendocrine tumor (NET)-related syndrome with variable presentations related to hormonal discharge and long-term complications, such as carcinoid heart disease (CHD). Carcinoid crisis (CC) is an acute and serious CS complication, due to a massive release of vasoactive hormones and biologically active substances, which may occur during stressogenic procedures such as peptide receptor radionuclide therapy (PRRT). CC is an emergency and life-threatening condition that needs to be prevented, promptly recognized, and managed. The purpose of this joint Italian multidisciplinary position paper is to support the involved personnel in delivering [177Lu]Lu-based PRRT to syndromic NET patients, focusing on CC management. Methods: The literature evidence regarding CS, CHD, and CC during/after PRRT and the knowledge of 22 Italian NET experts in Anesthesiology, Endocrinology, Gastroenterology, Nuclear Medicine, Oncology, and Surgery involved in PRRT were the basis for the present document. Results: The expert panel expressed several recommendations regarding: 1) CC high-risk patients identification; 2) CC prevention, including considerations of the behavioral and nutritional status, and somatostatin analogue therapy; 3) how to implement PRRT premedication in CC high-risk patients; 4) how to manage CC during PRRT, including post-PRRT monitoring, and radiation protection considerations. Conclusion: This practical guide summarizes the views and experience in CS, CHD, and CC in relation to PRRT of a joint NET-dedicated Italian multidisciplinary team. These recommendations should be taken into the context of Nuclear Medicine good practice and do not substitute any national and International legal or regulatory provisions.