ECE2013 Poster Presentations Endocrine tumours and neoplasia (66 abstracts)
Familial malignant paraganglioma is long-term stabilized with the tyrosine-kinase inhibitor sunitinib
Valeria Ramundo 1 , Francesca Marciello 1 , Michela Del Prete 1 , Vincenzo Marotta 1 , Raffaella Esposito 1 , Anna Chiara Carratù 1 , Chiara de Luca di Roseto 1 , Annamaria Colao 1 & Antongiulio Faggiano 1,
1Department of Clinical and Molecular Endocrinology and Oncology, “Federico II” University of Naples, Naples, Italy; 2Endocrinology, National Cancer Institute, “Fondazione G. Pascale”, Naples, Italy.
Introduction: Paragangliomas are neuroectodermal tumors that arise from adrenal medulla or extra-adrenal ganglia and are characterized by high vascularisation. A high rate of these tumours is genetically inherited. For malignant paragangliomas, chemo- and radio-therapy are potentially effective, but tumor response is of short duration and patient prognosis is quite poor. Sunitinib is a tyrosine-kinase inhibitor, targeting VEGFR1, -2, PDGFRα, -β, RET and c-Kit. Recent experiences highlighted that sunitinib is potentially effective in patients with malignant paraganglioma. Our data aim to suggest a long-term use of sunitinib to arrest progression in familial paragangliomas.
Case report: Two patients (female, 48 years; male, 27 years) affected with persistent post-surgical malignant abdominal paraganglioma associated with paragangliomatosis type 4 (SDHB mutations) were treated with sunitinib 37.5 mg/day. One of them experienced a partial response and one other a tumour stabilization. At the last evaluation, 42 months after starting sunitinib, biochemical parameters (24-hour urine catecholamines and metanephrines) were in the normality range and tumor lesions were stable. Sunitinib was well tolerated and no serious adverse event was observed in both patients.
Conclusions: Sunitinib seems to be an effective agent for the management of patients with unresectable/advanced familial malignant paragangliomas. The striking finding of this report is that sunitinib at the dose of 37.5 mg/day is able to obtain long-time stabilization of tumor progression.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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