Peptide receptor radionuclide therapy for metastatic paragangliomas
File(s)
Accepted version
Author(s)
Type
Journal Article
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 177Lu-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.
Date Issued
2016-05-01
Date Acceptance
2016-01-27
Citation
Medical Oncology, 2016, 33 (5)
ISSN
1559-131X
Publisher
Springer
Journal / Book Title
Medical Oncology
Volume
33
Issue
5
Copyright Statement
The final publication is available at Springer via https://dx.doi.org/10.1007/s12032-016-0737-9
Sponsor
Medical Research Council (MRC)
Grant Number
MR/N020782/1
Subjects
Science & Technology
Life Sciences & Biomedicine
Oncology
Malignant paraganglioma
Management
Peptide receptor radionuclide therapy
Survival
MALIGNANT PHEOCHROMOCYTOMAS
LU-177-DOTA(0),TYR(3) OCTREOTATE
NEUROENDOCRINE TUMORS
DIAGNOSIS
MANAGEMENT
SURVIVAL
Publication Status
Published
Article Number
47