Advances in the Management of Medullary Thyroid Carcinoma: Focus on Peptide Receptor Radionuclide Therapy.

Erika Grossrubatscher,Giuseppe Fanciulli,Federica De Cicco,Antongiulio Faggiano,Carlotta Dolci,Annamaria Colao,Luca Pes,Franz Sesti

doi:10.3390/jcm9113507

Abstract

Effective treatment options in advanced/progressive/metastatic medullary thyroid carcinoma (MTC) are currently limited. As in other neuroendocrine neoplasms (NENs), peptide receptor radionuclide therapy (PRRT) has been used as a therapeutic option in MTC. To date, however, there are no published reviews dealing with PRRT approaches. We performed an in-depth narrative review on the studies published in this field and collected information on registered clinical trials related to this topic. We identified 19 published studies, collectively involving more than 200 patients with MTC, and four registered clinical trials. Most cases of MTC were treated with PRRT with somatostatin analogues (SSAs) radiolabelled with 90 yttrium (90Y) and 177 lutetium (177Lu). These radiopharmaceuticals show efficacy in the treatment of patients with MTC, with a favourable radiological response (stable disease, partial response or complete response) in more than 60% of cases, coupled with low toxicity. As MTC specifically also expresses cholecystokinin receptors (CCK2Rs), PRRT with this target has also been tried, and some randomised trials are ongoing. Overall, PRRT seems to have an effective role and might be considered in the therapeutic strategy of advanced/progressive/metastatic MTC.

Highlights

Medullary thyroid carcinoma (MTC) is a rare neuroendocrine neoplasm (NEN)
We aimed to perform an in-depth narrative review on the studies published on peptide receptor radionuclide therapy (PRRT) and medullary thyroid carcinoma (MTC), in order to offer to the physician involved in the management of MTC an updated overview of the PRRT treatments
We found 18 PRRT studies targeting somatostatin receptor (SSR) involving patients with MTC: 14 with somatostatin analogue (SSA) labelled with the radionuclide(s) 90 yttrium (90Y) and/or 177 lutetium (177Lu) and four with SSA labelled with 111 indium (111In)

Summary

Introduction

Medullary thyroid carcinoma (MTC) is a rare neuroendocrine neoplasm (NEN). Surgery, with complete resection of the tumour, is, in most cases, curative [1]. In a subgroup of patients, the tumour shows an aggressive behaviour. In this setting (advanced/progressive/metastatic MTC), the management remains challenging [1,2]. Tyrosine kinase inhibitors (TKIs) have recently been shown to improve progression-free survival (PFS) in patients with advanced disease [3,4]. Two TKIs, namely vandetanib and cabozantinib, have been approved for the treatment of progressive or symptomatic MTC. Both drugs are effective, resulting in partial response (PR) or stable disease (SD), but tumour escape frequently occurs. As in other advanced/progressive/metastatic NENs, radionuclide therapy may represent a therapeutic option, and it has been applied in MTC since the 1980s. The first radiopharmaceutical used in therapy was 131Iodine (131I)-metaiodobenzylguanidine (MIBG) [5,6], but its use has been limited in MTC, due to the low percentage of MTC that shows uptake on MIBG scintigraphy [7,8,9]

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