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Distant metastases are the main cause of death in patients with medullary thyroid cancer (MTC). These 21 recommendations focus on MTC patients with distant metastases and a detailed follow-up protocol of patients with biochemical or imaging evidence of disease, selection criteria for treatment, and treatment modalities, including local and systemic treatments based on the results of recent trials. Asymptomatic patients with low tumor burden and stable disease may benefit from local treatment modalities and can be followed up at regular intervals of time. Imaging is usually performed every 6–12 months, or at longer intervals of time depending on the doubling times of serum calcitonin and carcinoembryonic antigen levels. Patients with symptoms, large tumor burden and progression on imaging should receive systemic treatment. Indeed, major progress has recently been achieved with novel targeted therapies using kinase inhibitors directed against RET and VEGFR, but further research is needed to improve the outcome of these patients.
UMR9019, Genome Integrity and Cancers, CNRS, Villejuif, France
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CHU Nantes/ICO, Saint-Herblain, France
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CHU Nantes/ICO, Saint-Herblain, France
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Université de Paris, Paris, France
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Department of Radiology, AP-HP, Hôpital Cochin, Paris, France
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Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy Institute, Villejuif, France
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Department of Medical Oncology, AP-HP, Hôpital Cochin, Paris, France
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Université de Paris, Paris, France
Department of Endocrinology, AP-HP, Hôpital Cochin, Paris, France
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Background: Cardiac metastases from thyroid cancers are uncommon with a poor prognosis. There is a lack of long-term follow-up studies. Cases: We report 2 cases of cardiac metastasis from medullary thyroid cancer (MTC). Both patients presented limited metastatic disease apart from a cardiac metastasis. The initial diagnosis was challenging and was facilitated by functional imaging with an immuno-PET-CT using an anti-CEA bispecific antibody and a <sup>68</sup>Ga-labeled peptide. Both patients were treated with the multitarget kinase inhibitor vandetanib with prolonged stability. The first patient was alive at the last follow-up, 14 years after the diagnosis of cardiac metastasis. The second patient required surgical excision of the cardiac mass because of disease progression under vandetanib. Conclusion: These cases illustrate long-term survival and effectiveness of clinical management of 2 patients who developed cardiac metastases from MTC, in the current era of personalized medicine with targeted therapy.
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in the remaining 25%, in the context of multiple endocrine neoplasia 2 (MEN2) syndrome, due to a germline REarranged during Transfection mutation. MTC is treated with a total thyroidectomy and a central lymph node dissection. More extensive surgery is
