Efficacy and Safety of Ablative Therapy in the Treatment of Patients with Metastatic Pheochromocytoma and Paraganglioma.

Jacob Kohlenberg,Brian Welch,Irina Bancos,Oksana Hamidi,Juraj Sprung,Jonathan Morris,Matthew Callstrom,William Young

doi:10.3390/cancers11020195

Abstract

Metastatic pheochromocytoma and paraganglioma (PPGL) are incurable neuroendocrine tumors. The goals of treatment include palliating symptoms and reducing tumor burden. Little is known about the use of radiofrequency ablation (RFA), cryoablation (CRYO), and percutaneous ethanol injection (PEI) to treat metastatic PPGL. We performed a retrospective study of patients age 17 years and older with metastatic PPGL who were treated with ablative therapy at Mayo Clinic, USA, between June 14, 1999 and November 14, 2017. Our outcomes measures were radiographic response, procedure-related complications, and symptomatic improvement. Thirty-one patients with metastatic PPGL had 123 lesions treated during 42 RFA, 23 CRYO, and 4 PEI procedures. The median duration of follow-up was 60 months (range, 0–163 months) for non-deceased patients. Radiographic local control was achieved in 69/80 (86%) lesions. Improvement in metastasis-related pain or symptoms of catecholamine excess was achieved in 12/13 (92%) procedures. Thirty-three (67%) procedures had no known complications. Clavien-Dindo Grade I, II, IV, and V complications occurred after 7 (14%), 7 (14%), 1 (2%), and 1 (2%) of the procedures, respectively. In patients with metastatic PPGL, ablative therapy can effectively achieve local control and palliate symptoms.

Highlights

Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare neuroendocrine tumors that arise from the adrenal medulla and autonomic paraganglia, respectively
We found that ablative therapy can be successfully used in patients with metastatic pheochromocytoma and paraganglioma (PPGL) to achieve radiographic local control, palliate metastasis-related pain, and reduce symptoms of catecholamine excess
We found that the main indications for ablation therapy were to palliate metastasis-related pain, reduce manifestations of catecholamine excess, stabilize metastases in high-risk anatomic locations, and achieve local oncologic control to prevent risks associated with local tumor progression

Summary

Introduction

Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare neuroendocrine tumors that arise from the adrenal medulla and autonomic paraganglia, respectively. The incidence in the United States is 500 to 1600 cases per year [1]. While the majority of PHEO and PGL (PPGL) do not metastasize, 2–13% of PHEO and 2.4–50% of PGL are metastatic [2,3,4]. PPGL are considered metastatic when nodal or distant metastases are identified [5]. Patients with metastatic PPGL most frequently present with manifestations of catecholamine excess [6]. Patients present with symptoms from tumor-related mass effect or incidentally following imaging performed for an unrelated indication [6]. The natural history of metastatic PPGL is highly variable: for some patients it progresses rapidly, but others have prolonged

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