Peptide receptor radionuclide therapy for metastatic paragangliomas

Abstract

There is little evidence to direct the management of malignant paragangliomas (mPGL) beyond initial surgical treatment. Peptide receptor radionuclide therapy (PRRT), using somatostatin analogues, is effective in other neuroendocrine tumours, but data on its efficacy in treating mPGL are scarce. We report safety and efficacy outcomes from a case series of five patients with advanced mPGLs treated with (177)Lu-DOTATATE PRRT. The mean age of our cohort was 34 years (range 16-47); 4 patients were male with bone disease being the most prevalent metastatic site. PRRT scheme varied between 1 and 4 cycles, with premature cessation due to suspected pneumonitis in one case and disease progression in another. Three patients with previously documented progressive disease achieved stabilization following treatment; one had partial response and one was treatment refractory. Median progression-free survival was 17 months (range 0-78 months). 177-Lu-DOTATATE is an effective therapy in mPGLs in this molecularly defined patient cohort, warranting further investigation in larger studies including hereditary and sporadic mPGL.

Pheochromocytoma: A Genetic And Diagnostic Update

“…SSTR overexpression in PPGL led to their use as therapeutic targets. By attaching radionuclides ( 177 Lu, 90 Y, 111 In) via a linker and vector mechanism to a targeting moiety (DOTATATE, DOTANOC, DOTATOC), PRRT has been studied in well-differentiated, advanced neuroendocrine tumors (NETs) (83)(84)(85)(86)(87). While overall results have varied, the majority of studies report the feasibility, effectiveness, and efficacy of PRRT in NETs (84,87), with increased progression free survival (PFS), as well as patient reported pain relief, symptom regression, and increased quality of life (83).…”

‘Peptide receptor radionuclide therapy in the management of advanced pheochromocytoma and paraganglioma: A systematic review and meta‐analysis’

“…Though the experience with PRRT is predominantly limited to gastroenteropancreatic neuroendocrine tumours, few studies have also evaluated its efficacy and safety in PPGLs . However, the individual studies have been conducted with limited number of patients and the precise effect of PRRT in treatment of advanced PPGLs is currently unclear.…”

Eruption of Metastatic Paraganglioma After Successful Therapy with 177Lu/90Y-DOTATOC and 177Lu-DOTATATE

“…Previous studies have reported significant therapeutic responses and increased progression free survival in patients with various neuroendocrine tumors, including metastatic PHEO/PGL, undergoing PRRT [6,[10][11][12][13][14][15]. Currently, PRRT is performed according to different protocols: a combination of 90 Y-DOTATOC and 177 Lu-DOTATOC or monotherapy with 1 77 Lu-DOTATATE.…”