title: Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre.
abstract: BACKGROUND
  Phaeochromocytoma (phaeo) and paraganglioma (PGL) are rare conditions, which are malignant in up to 30%. Optimal treatment is controversial, but in patients with metastatic iodine-131-meta-iodobenzylguanidine ((123)I-MIBG) avid tumours, we offer (131)I-MIBG therapy. We summarize response rates, survival and safety in a cohort of such patients treated with (131)I-MIBG in our centre from 1986 to 2012.
  
  
  METHODS
  Retrospective analysis of the case notes of patients with metastatic phaeo/PGL who received (131)I-MIBG was undertaken; patients underwent clinical, biochemical and radiological evaluation within 6 months of each course of (131)I-MIBG therapy.
  
  
  RESULTS
  Twenty-two patients (9 males) were identified, 12 with metastatic PGL and 10 with phaeo. Overall median follow-up time after first dose of (131)I-MIBG was 53 months. In total, 68 doses of (131)I-MIBG were administered; average dose was 9967 MBq (269.4 mCi). After the first dose, >50% of patients demonstrated disease stability or partial response; progressive disease was seen in 9%. A subset of patients underwent repeated treatment with the majority demonstrating partial response or stable disease. No life-threatening adverse events were reported, but three patients developed hypothyroidism and two developed ovarian failure after repeated dosing. Five-year survival after original diagnosis was 68% and median (+inter quartile range) survival from date of diagnosis was 17 years (7.6-26.4) with no difference in survival according to diagnosis (P < 0.1).
  
  
  CONCLUSIONS
  (131)I-MIBG is well tolerated and associates with disease stabilization or improvement in the majority of patients with metastatic phaeo/PGL. However, stronger conclusions on treatment effectiveness are limited by lack of a directly comparable 'control group' as well as an alternative 'gold standard' treatment.
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK.
authors.forename: M A
authors.initials: MA
authors.lastname: Rutherford
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK.
authors.forename: A J
authors.initials: AJ
authors.lastname: Rankin
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK.
authors.forename: T M
authors.initials: TM
authors.lastname: Yates
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK.
authors.forename: P B
authors.initials: PB
authors.lastname: Mark
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK.
authors.forename: C G
authors.initials: CG
authors.lastname: Perry
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK.
authors.forename: N S
authors.initials: NS
authors.lastname: Reed
authors.affiliation: From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK From the Department of Endocrinology, Western Infirmary, Dumbarton Road, Glasgow G11 6NT, UK, Institute of Cardiovascular & Medical Sciences, University of Glasgow, 126 University Place, Glasgow G12 8TA, UK and Beatson West of Scotland Oncology Centre, Gartnavel General Hospital, 1053 Great Western Road, Glasgow G12 0YN, UK Marie.Freel@glasgow.ac.uk.
authors.forename: E M
authors.initials: EM
authors.lastname: Freel
mesh_terms: D019797:3-Iodobenzylguanidine / Q000008:administration & dosage* / Q000009:adverse effects; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000532:radiotherapy* / Q000556:secondary; D000328:Adult; D000368:Aged; D019468:Disease Management; D005260:Female; D005820:Genetic Testing; D006801:Humans; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000378:metabolism / Q000532:radiotherapy*; D010673:Pheochromocytoma / Q000378:metabolism / Q000532:radiotherapy*; D019275:Radiopharmaceuticals / Q000008:administration & dosage* / Q000009:adverse effects; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine