Overview

NCI Definition: 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. [1]

Thyroid gland medullary carcinomas most frequently harbor alterations in RET, HRAS, KRAS, and KMT2D [2].

Most Commonly Altered Genes in Thyroid Gland Medullary Carcinoma

RET Mutation, RET M918T, HRAS Mutation, HRAS Missense, and KRAS Mutation are the most common alterations in thyroid gland medullary carcinoma [2].

Top Alterations in Thyroid Gland Medullary Carcinoma

Significant Genes in Thyroid Gland Medullary Carcinoma

AXL +

MET +

RET +

Disease Details

Synonyms
MTC, Parafollicular Cell Carcinoma, Thyroid Gland Neuroendocrine Carcinoma, Medullary Thyroid Gland Carcinoma, Thyroid Medullary Carcinoma, Medullary Carcinoma, C Cell Carcinoma, Medullary Carcinoma of Thyroid, Medullary Carcinoma of the Thyroid Gland, CARCINOMA, C-CELL, MALIGNANT, Medullary Carcinoma of Thyroid Gland, Medullary Thyroid Carcinoma, Medullary Thyroid Cancer, Medullary Carcinoma of the Thyroid
Parent(s)
Thyroid Gland Carcinoma
OncoTree Name
Medullary Thyroid Cancer
OncoTree Code
THME

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.