title: 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.
authors.affiliation: Division of Surgery and Cancer, Imperial College London, Hammersmith Hospital, Du Cane Road, London, W120HS, UK.
authors.forename: David J
authors.initials: DJ
authors.lastname: Pinato
authors.affiliation: Division of Surgery and Cancer, Imperial College London, Hammersmith Hospital, Du Cane Road, London, W120HS, UK.
authors.forename: James R M
authors.initials: JR
authors.lastname: Black
authors.affiliation: Department of Medical Oncology, Imperial College London, Hammersmith Hospital, Du Cane Road, London, W120HS, UK.
authors.forename: Ramya
authors.initials: R
authors.lastname: Ramaswami
authors.affiliation: Division of Diabetes, Endocrinology and Metabolism, Department of Investigative Medicine, Imperial College London, London, UK.
authors.forename: Tricia M
authors.initials: TM
authors.lastname: Tan
authors.affiliation: Department of Medical Oncology, Imperial College London, Hammersmith Hospital, Du Cane Road, London, W120HS, UK.
authors.forename: Delali
authors.initials: D
authors.lastname: Adjogatse
authors.affiliation: Division of Surgery and Cancer, Imperial College London, Hammersmith Hospital, Du Cane Road, London, W120HS, UK. r.sharma@imperial.ac.uk.|Division of Oncology and Clinical Pharmacology, Imperial College London, Hammersmith Campus, Du Cane Road, London, W120HS, UK. r.sharma@imperial.ac.uk.
authors.forename: Rohini
authors.initials: R
authors.lastname: Sharma
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008187:Lutetium / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D015282:Octreotide / Q000031:analogs & derivatives / Q000627:therapeutic use; D009942:Organometallic Compounds / Q000627:therapeutic use; D010235:Paraganglioma / Q000401:mortality / Q000473:pathology* / Q000532:radiotherapy*; D011868:Radioisotopes / Q000627:therapeutic use; D011878:Radiotherapy / Q000379:methods*; D016896:Treatment Outcome
chemical_list: D009942:Organometallic Compounds; D011868:Radioisotopes; D008187:Lutetium; C447941:lutetium Lu 177 dotatate; D015282:Octreotide
keywords: Malignant paraganglioma; Management; Peptide receptor radionuclide therapy; Survival