Management of metastatic phaeochromocytoma and paraganglioma: use of Iodine-131-meta-iodobenzylguanidine (131I-MIBG) therapy in a tertiary referral centre

Rutherford, M.A., Rankin, A.J. , Yates, T.M., Mark, P.B. , Perry, C.G., Reed, N.S. and Freel, E.M. (2015) Management of metastatic phaeochromocytoma and paraganglioma: use of Iodine-131-meta-iodobenzylguanidine (131I-MIBG) therapy in a tertiary referral centre. QJM: An International Journal of Medicine, 108(5), pp. 361-368. (doi: 10.1093/qjmed/hcu208) (PMID:25267727)

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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 (123I-MIBG) avid tumours, we offer 131I-MIBG therapy. We summarize response rates, survival and safety in a cohort of such patients treated with 131I-MIBG in our centre from 1986 to 2012. Design/Methods: Retrospective analysis of the case notes of patients with metastatic phaeo/PGL who received 131I-MIBG was undertaken; patients underwent clinical, biochemical and radiological evaluation within 6 months of each course of 131I-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 131I-MIBG was 53 months. In total, 68 doses of 131I-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: 131I-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.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Reed, Dr Nicholas and Freel, Dr Marie and Mark, Professor Patrick and Perry, Dr Colin and Rankin, Dr Alastair
Authors: Rutherford, M.A., Rankin, A.J., Yates, T.M., Mark, P.B., Perry, C.G., Reed, N.S., and Freel, E.M.
Subjects:R Medicine > R Medicine (General)
College/School:College of Medical Veterinary and Life Sciences > School of Cardiovascular & Metabolic Health
College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
Journal Name:QJM: An International Journal of Medicine
Publisher:Oxford University Press
ISSN:1460-2725
ISSN (Online):1460-2393
Published Online:29 September 2014

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
496251Translational studies on the role of corticosteroids in cardiovascular diseaseMarie FreelMedical Research Council (MRC)G0802803RI CARDIOVASCULAR & MEDICAL SCIENCES