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A Journal on Otorhinolaryngology, Head and Neck Surgery,
Plastic Reconstructive Surgery, Otoneurosurgery

Indexed/Abstracted in: EMBASE, Scopus




Otorinolaringologia 2016 June;66(2):31-43

language: English

An overview of medullary thyroid carcinoma

Carol LI, Tiffany PENG, David I. KUTLER

Department of Otolaryngology, Head and Neck Surgery, New York Presbyterian Hospital/Weill Cornell Medical College, New York, NY, USA


Medullary thyroid carcinoma (MTC) is a malignant tumor of parafollicular or C-cell differentiation. It is the third most common thyroid cancer after papillary and follicular carcinomas and accounts for 1-2% of thyroid cancers in the United States. MTC exists in both sporadic and hereditary forms, as part of multiple endocrine neoplasia type 2 (MEN2), an autosomal dominant cancer syndrome. Hereditary MTC occurs due to gain-of-function mutations in the RET proto-oncogene. Different mutations portend specific phenotypes and can be stratified into low, moderate, and high-risk groups, with implications for screening and therapeutic interventions. Diagnosis of MTC utilizes multimodal techniques involving histopathological analysis, measurement of secretory products, radiographic investigation, and finally, genetic testing. Surgical management of MTC consists of total thyroidectomy with cervical lymph node dissection but is largely dictated by preoperative evaluation. In cases of hereditary syndromes, prophylactic thyroidectomy is recommended in general but timing and type of surgery varies on the underlying syndrome, biochemical markers, and genetic testing results. Serum measurements of secretory products, such as calcitonin and carcinoembryonic antigen, not only have a major role in screening and diagnosis of MTC, but also in the postoperative assessment of surgical cure and disease surveillance. In addition to surgery, various systemic therapies to address advanced MTC, such as tyrosine kinase inhibitors, are currently under investigation.

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