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Medullary Thyroid Cancer

  • Rossella Elisei
  • Cristina Romei
Chapter

Abstract

Medullary thyroid cancer is very rare since it represents about 0.2% of all human tumors. It can be sporadic (75%) or familial (25%), and in both cases the pathogenesis is related to the presence of activating mutations of RET oncogene at somatic or germline level, respectively. Total thyroidectomy and central neck nodes dissection is the treatment of choice even when the tumor is apparently restricted to the thyroid. Lateral neck dissection should be reserved to cases with an evident involvement of lymph nodes at neck ultrasound. The initial treatment can be curative only if the tumor is still intrathyroid or if few and small lymph node metastases in the central neck compartment are present. The presence of distant metastases at the time of diagnosis is the poorest prognostic factor for both the cure and survival of patients. Very high levels of presurgical serum calcitonin, the marker of the disease, particularly if accompanied by elevated levels of carcinoembryonic antigen, should suggest to perform a computerized tomography scan of the neck and chest to better plan the surgical treatment. Metastatic disease must be treated when lesions are progressing or putting some vital organs at risk of compression or infiltration. A local treatment, such as thermoablation or chemoembolization or external radiation treatment, should always be considered as first approach. When the metastatic lesions are multiple, located in different organs and growing, a systemic therapy with the new targeted drugs inhibiting tyrosine kinase receptors should be started.

Keywords

Calcitonin RET Medullary thyroid cancer Vandetanib Cabozantinib 

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© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Endocrine Unit, Department of Clinical and Experimental MedicineUniversity Hospital of PisaPisaItaly

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