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Objectives  Inoperable or metastatic paragangliomas (PGLs) and malignant pheochromocytomas (PCCs) are rare tumours with limited options for systemic treatment. Aim of this study was to assess the safety and efficacy of the radiolabelled somatostatin analogue ( 177 LutetiumDOTA 0 -Tyr 3 )octreotate ( 177 Lu-DOTATATE) for the treatment of PGLs and PCCs.    Methods  Patients with histologically proven inoperable or malignant PGLs and PCCs treated with  177 Lu-DOTATATE at our centre were retrospectively analysed. Patients were treated with up to four cycles of  177 Lu-DOTATATE with an intended dose of 7.4 Gb per cycle. Response was assessed with use of RECIST 1.1.    Results  Thirty patients were included: 17 with parasympathetic, 10 with sympathetic PGLs and 3 with PCCs. Grade 3/4 subacute haematotoxicity occurred in 6 (20%) of patients. A reversible subacute adverse event due to cardiac failure following possible catecholamine release occurred in two patients. Best tumour response was partial response in 7 (23%) and stable disease in 20 (67%), whereas 3 (10%) patients had progressive disease. In 20 patients with baseline disease progression, tumour control was observed in 17 (85%); the median progression-free survival was 91 months in patients with parasympathetic PGLs, 13 months in patients with sympathetic PGLs and 10 months in patients with metastatic PCCs.    Conclusion  This study suggests that PRRT with  177 Lu-DOTATATE is a safe and effective treatment option for patients with inoperable or malignant PGL and PCC.

Treatment of inoperable or metastatic paragangliomas and pheochromocytomas with peptide receptor radionuclide therapy using 177Lu-DOTATATE