title: Current understanding and management of medullary thyroid cancer.
abstract: Medullary thyroid cancer (MTC) typically accounts for 3%-4% of all thyroid cancers. Although the majority of MTCs are sporadic, 20% of cases are hereditary. Hereditary MTC can be found in multiple endocrine neoplasia 2A or 2B or as part of familial MTC based on a specific germline mutation in the RET proto-oncogene. This article discusses the current approaches available for the diagnosis, evaluation, and management of patients and their family members with suspected MTC. The disease is predominantly managed surgically and typically requires a total thyroidectomy and lymph node dissection. A review of recent guidelines on the extent and timing of surgical excision is discussed. There are not very many effective systemic treatment options for MTC, but several emerging therapeutic targets have promise.
authors.affiliation: Department of Surgery, University of Wisconsin, Madison, Wisconsin, USA.
authors.forename: Madhuchhanda
authors.initials: M
authors.lastname: Roy
authors.forename: Herbert
authors.initials: H
authors.lastname: Chen
authors.forename: Rebecca S
authors.initials: RS
authors.lastname: Sippel
mesh_terms: D002116:Calcitonin / Q000097:blood; D018276:Carcinoma, Medullary / Q000097:blood / Q000151:congenital* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D018278:Carcinoma, Neuroendocrine; D018095:Germ-Line Mutation; D006801:Humans; D008197:Lymph Node Excision; D018813:Multiple Endocrine Neoplasia Type 2a / Q000097:blood / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D000090063:Proto-Oncogene Mas; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics*; D013964:Thyroid Neoplasms / Q000097:blood / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D013965:Thyroidectomy
chemical_list: C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D002116:Calcitonin; D051096:Proto-Oncogene Proteins c-ret; C099282:RET protein, human
keywords: Familial Medullary Thyroid Cancer (FMTC); Medullary Thyroid Cancer (MTC); Multiple Endocrine Neoplasia Syndrome; Prophylactic surgery in MTC; RET proto-oncogene; Serum calcitonin
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