title: Surgical and clinical strategies in the management of thyroid medullary carcinoma in children with and without ret proto-oncogene mutations.
abstract: Medullary thyroid carcinoma (MTC) may arise sporadically or in familial manner. We presented sporadic and familial cases with MTC in order to raise awareness on management of such patients. Three medullary thyroid carcinoma (MTC) cases were presented. Case 1 had RET634 mutation; managed with total thyroidectomy (TT) and cervical lymph node dissection (CLND). Case 2 had RET804 mutation; managed with prophylactic TT. Case 3 had thyroid nodule; managed with TT and CLND. Case 1 had micro-carcinomatosis foci, Case 2 had normal thyroid tissue in histopathological examination and Case 3 had medullary thyroid carcinoma with tumor negative surgical borders. Case 1 was re-operated for persisting focus of disease. Follow-up of cases were uneventful. Clinicians and surgeons should be aware of critical timing for surgery and various surgical and clinical strategies in the management of MTC in children.
authors.affiliation: Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Özlem
authors.initials: Ö
authors.lastname: Boybeyi-Türer
authors.affiliation: Divisions of Pediatric Endocrinology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Doğuş
authors.initials: D
authors.lastname: Vurallı
authors.affiliation: Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: İbrahim
authors.initials: İ
authors.lastname: Karnak
authors.affiliation: Divisions of Pediatric Endocrinology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Nazlı
authors.initials: N
authors.lastname: Gönç
authors.affiliation: Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Emel Şule
authors.initials: EŞ
authors.lastname: Yalçın
authors.affiliation: Pediatric Pathology, Department of Pediatrics, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Diclehan
authors.initials: D
authors.lastname: Orhan
authors.affiliation: Divisions of Pediatric Endocrinology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Nurgün
authors.initials: N
authors.lastname: Kandemir
authors.affiliation: Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
authors.forename: Feridun Cahit
authors.initials: FC
authors.lastname: Tanyel
mesh_terms: D000293:Adolescent; D018278:Carcinoma, Neuroendocrine / Q000601:surgery*; D002648:Child; D006801:Humans; D008197:Lymph Node Excision / Q000379:methods; D008297:Male; D018813:Multiple Endocrine Neoplasia Type 2a / Q000601:surgery*; D009154:Mutation; D000090063:Proto-Oncogene Mas; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics*; D013964:Thyroid Neoplasms / Q000601:surgery*; D013965:Thyroidectomy / Q000379:methods*
chemical_list: C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D051096:Proto-Oncogene Proteins c-ret
keywords: RET proto-oncogene; medullary thyroid carcinoma; multiple endocrine neoplasia; surgery