medullary thyroid gland carcinoma
MONDO:0015277
A neuroendocrine carcinoma arising from the C-cells of the thyroid gland. It is closely associated with multiple endocrine neoplasia syndromes. Approximately 10% to 20% of medullary thyroid carcinomas are familial. Patients usually present with a thyroid nodule that is painless and firm. In the majority of cases nodal involvement is present at diagnosis. Surgery is the preferred treatment for both primary lesions and recurrences. This carcinoma is generally not very sensitive to radiation and almost unresponsive to chemotherapy.
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Medullary thyroid cancer
Medullary thyroid cancer is a form of thyroid carcinoma which originates from the parafollicular cells (C cells), which produce the hormone calcitonin. Medullary tumors are the third most common of all thyroid cancers and together make up about 3% of all thyroid cancer cases. MTC was first characterized in 1959. Approximately 25% of medullary thyroid cancer cases are genetic in nature, caused by a mutation in the RET proto-oncogene. When MTC occurs by itself it is termed sporadic medullary thyroid cancer. Medullary thyroid cancer is seen in people with multiple endocrine neoplasia type 2, subtypes 2A and 2B. When medullary thyroid cancer due to a hereditary genetic disorder occurs without other endocrine tumours it is termed familial medullary thyroid cancer.
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Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.