malignant pheochromocytoma

clinical endocrinology (oxf)

Clin Endocrinol (Oxf). 2013 Oct 1.
131 I-MIBG therapyfor Malignant Paraganglioma and Pheochromocytoma: Systematic Review and Meta-Analysis.
van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP.
Source
Departments of Endocrinology and Metabolic Diseases, Leiden University Medical Center, Leiden, the Netherlands.

Abstract
BACKGROUND:
131 I-MIBG therapy can be used for palliative treatment of malignant paraganglioma and pheochromocytoma. The main objective of the present study was to perform a systematic review and meta-analysis assessing the effect of 131 I-MIBG therapy on tumor volume in patients with malignant paraganglioma/pheochromocytoma.

METHODS:
A literature search was performed in December 2012 to identify potentially relevant studies. Main outcomes were the pooled proportions of complete response, partial response and stable disease after radionuclide therapy. A meta-analysis was performed with an exact likelihood approach using a logistic regression with a random effect at the study level. Pooled proportions with 95% confidence intervals (CI) were reported.

RESULTS:
Seventeen studies concerning a total of 243 patients with malignant paraganglioma/pheochromocytoma were treated with 131 I-MIBG therapy. The mean follow-up ranged from 24 to 62 months. A meta-analysis of the effect of 131 I-MIBG therapy on tumor volume showed pooled proportions of complete response, partial response and stable disease of respectively 0.03 (95% CI 0.06-0.15), 0.27 (95% CI 0.19-0.37) and 0.52 (95% CI 0.41-0.62) and for hormonal response 0.11 (95% CI 0.05-0.22), 0.40 (95% CI 0.28-0.53) and 0.21 (95% CI 0.10-0.40), respectively.Separate analyses resulted in better results in hormonal response for paraganglioma patients than for pheochromocytoma patients.

CONCLUSIONS:
Data on the effects of 131 I-MIBG therapy on malignant paraganglioma/pheochromocytoma suggest that stable disease concerning tumor volume and a partial hormonal response can be achieved in over 50% and 40% of patients respectively, treated with 131 I-MIBG therapy. It cannot be ruled out that stable disease reflects not only the effect of MIBG-therapy, but also(partly) the natural course of the disease. This article is protected by copyright. All rights reserved.
This article is protected by copyright. All rights reserved.
KEYWORDS:
131I-MIBG therapy, hormonal response, paraganglioma, peptide receptor radionuclide therapy, pheochromocytoma, radionuclide therapy, survival, systematic review, toxicity, tumor response
PMID:
24118038