Supportive Management of Patients with Advanced Pheochromocytomas and Paragangliomas Receiving PRRT.

Erica S Tsang,Janet Leung,Hagen F Kennecke,Gayle Funk,Grace Kalish

doi:10.3390/curroncol28040247

Abstract

Peptide receptor radionuclide therapy (PRRT) is used to treat patients with advanced malignant pheochromocytomas (PCCs) and paragangliomas (PGLs). Patients are at risk of a PRRT-induced catecholamine crisis, and standard guidelines regarding the prevention and management of infusion reactions are lacking. In this case series, the institutional experience of five sequential patients with metastatic PCCs and PGLs receiving PRRT on an outpatient basis is described, of which four had symptomatic tumors and three had a high burden of disease. All patients with symptomatic tumors were treated with preventive management prior to the initiation of PRRT, and no infusion reactions or catecholamine crises were documented. PRRT may be delivered safely on an outpatient basis for patients with metastatic PCCs and PGLs with the involvement of an interdisciplinary team.

Highlights

Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent rare neuroendocrine tumors with variable 5-year survival outcomes in the metastatic setting, ranging from 34–69% [1]
peptide receptor radionuclide therapy (PRRT) has been approved in Canada for patients with unresectable midgut NETs after progression on a somatostatin analogue
The components of PRRT, including the radionuclide Lutetium-177, peptide, and chelator DOTA, bind to somatostatin receptors on the PCC or PGL tumor cell membrane. This subsequently leads to internalization and local delivery of beta-radiation from Lutetium-177, causing cell damage and death [10]

Summary

Introduction

Pheochromocytomas (PCCs) and paragangliomas (PGLs) represent rare neuroendocrine tumors with variable 5-year survival outcomes in the metastatic setting, ranging from 34–69% [1]. In 2018, peptide receptor radionuclide therapy (PRRT) was approved in the United States for the treatment of advanced gastroenteropancreatic neuroendocrine tumors (GEP-NETs) with positive expression of somatostatin receptors [8]. The components of PRRT, including the radionuclide Lutetium-177, peptide, and chelator DOTA, bind to somatostatin receptors on the PCC or PGL tumor cell membrane. This subsequently leads to internalization and local delivery of beta-radiation from Lutetium-177, causing cell damage and death [10]. PRRT protocol modifications, including lengthening infusion time and decreasing upfront treatment dosage, have been suggested to mitigate or prevent adverse reactions, such as a catecholamine crisis or tumor lysis syndrome [17].

Case 1

Case 4

Findings

Case 5