title: Medullary thyroid carcinoma: including MEN 2A and MEN 2B syndromes.
abstract: Medullary thyroid carcinoma (MTC) is a rare malignancy with several distinctive features that distinguish its management from other thyroid cancers. First, MTC may be sporadic (75% of cases), or may occur as a manifestation of the hereditary syndrome Multiple Endocrine Neoplasia type 2 (MEN 2) (25% of cases). Additionally, while MTC is more difficult to cure than differentiated thyroid cancer and has higher rates of recurrence and mortality, it is usually a slow growing tumor compared with other malignancies. Finally, unlike differentiated thyroid cancer, there is no known effective systemic therapy for MTC. MTC cells do not concentrate radioactive iodine, and MTC does not respond well to external beam radiation or conventional cytotoxic chemotherapy. These distinguishing features should be considered in planning surgical management of MTC.
authors.affiliation: Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Frank J
authors.initials: FJ
authors.lastname: Quayle
authors.forename: Jeffrey F
authors.initials: JF
authors.lastname: Moley
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002116:Calcitonin / Q000097:blood; D018276:Carcinoma, Medullary / Q000188:drug therapy / Q000235:genetics / Q000532:radiotherapy / Q000601:surgery; D003131:Combined Modality Therapy; D003606:Dacarbazine / Q000008:administration & dosage; D013507:Endocrine Surgical Procedures / Q000379:methods; D005472:Fluorouracil / Q000008:administration & dosage; D006801:Humans; D008197:Lymph Node Excision; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics / Q000473:pathology; D018814:Multiple Endocrine Neoplasia Type 2b / Q000235:genetics / Q000473:pathology; D009154:Mutation; D016105:Parathyroidectomy; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D013964:Thyroid Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000532:radiotherapy / Q000601:surgery; D013965:Thyroidectomy; D014182:Transplantation, Autologous
chemical_list: D011518:Proto-Oncogene Proteins; D003606:Dacarbazine; D002116:Calcitonin; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases; D005472:Fluorouracil