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- Title
Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre.
- Authors
RUTHERFORD, M. A.; RANKIN, A. J.; YATES, T. M.; MARK, P. B.; PERRY, C. G.; REED, N. S.; FREEL, E. M.
- 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 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 6 8% 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.
- Subjects
PHEOCHROMOCYTOMA; PARAGANGLIOMA; IODINE isotopes; GUANIDINES; METASTASIS; TERTIARY care; RARE diseases; THERAPEUTICS
- Publication
QJM: An International Journal of Medicine, 2015, Vol 108, Issue 5, p361
- ISSN
1460-2725
- Publication type
Article
- DOI
10.1093/qjmed/hcu208