Medullary thyroid cancer.
Medullary thyroid cancer is a rare neoplasm that arises from the parafollicular C cells. It occurs in a sporadic form, or less commonly as a hereditary form, as part of multiple endocrine neoplasia syndromes types 2A and 2B. The RET proto-oncogene is currently the primary factor that is implicated in the hereditary forms of this neoplasm. The knowledge about the genetic makeup of the neoplasm impacts upon management as it allows for screening, early detection, and prophylactic treatment. Surgery is the main modality that offers a cure. This entails a total thyroidectomy and vigilant management and surveillance of the neck. Prognosis of patients with MTC is variable, but the more constant factors that affect it are the stage of disease and the age of the patient. The emerging molecular genetic understanding of this malignancy will provide the foundation for prognostic and therapeutic decision-making in the future. Interdisciplinary management by surgeons, endocrinologists, pathologists, radiotherapists, radiologists, and medical oncologists should be sought.
Clayman
Gary L
GL
Department of Head and Neck Surgery, University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Box 0441, Houston, TX 77030, USA. gclayman@mdanderson.org
el-Baradie
Tarek S
TS
D014408:Biomarkers, Tumor; D018276:Carcinoma, Medullary / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D009367:Neoplasm Staging; D011237:Predictive Value of Tests; D011379:Prognosis; D000090063:Proto-Oncogene Mas; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D013964:Thyroid Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*
D014408:Biomarkers, Tumor; C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D011518:Proto-Oncogene Proteins; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases