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.