Medullary Thyroid Carcinoma

  • Martha B. Pitman
  • Yolanda C. Oertel
  • Kim R. Geisinger


In 1951 Horn described and illustrated what is now known as medullary thyroid carcinoma (MTC). A few years later, Hazard and colleagues reported 21 cases of “this medullary or solid form of thyroid carcinoma” and recommended that it be recognized as a distinct clinicopathologic entity. In 1966, Williams suggested that it was derived from the calcitonin-producing “C cells” or parafollicular cells of the thyroid. A few years later, investigators showed conclusively that MTCs were immunoreactive for calcitonin.

MTC comprises approximately 7% of thyroid carcinomas. It occurs in sporadic (in 85% of cases) and heritable forms. The heritable forms include multiple endocrine neoplasia (MEN) type 2A (Sipple’s syndrome), which includes pheochromocytomas and hyperparathyroidism in some families; MEN type 2B (mucosal neuroma syndrome or Gorlin’s syndrome), which consistently includes mucosal neuromas and somatic marfanoid habitus; and familial medullary thyroid carcinoma (FMTC). The hereditary forms are characterized by an autosomal dominant mode of inheritance and are associated with point mutations in the RET proto-oncogene on chromosome 10.


Papillary Carcinoma Medullary Thyroid Carcinoma Autosomal Dominant Mode Familial Medullary Thyroid Carcinoma Parafollicular Cell 
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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Martha B. Pitman
    • 1
  • Yolanda C. Oertel
    • 2
  • Kim R. Geisinger
    • 3
  1. 1.Department of PathologyMassachusetts General Hospital and Harvard Medical SchoolBostonUSA
  2. 2.Department of PathologyWashington Hospital CenterWashingtonUSA
  3. 3.Department of PathologyWake Forest University School of MedicineWinston-SalemUSA

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