title: Surgery in MEN 2A Patients Older Than 5 Years with Micro-MTC: Outcome at Long-term Follow-up.
abstract: In multiple endocrine neoplasia syndrome type 2A (MEN 2A), early total thyroidectomy (TT; performed before the age of 5 years) is the best option to prevent medullary thyroid carcinoma (MTC) development, but the management of MEN 2A patients diagnosed after childhood is still under debate. Seventeen consecutive patients diagnosed with MEN 2A after the age of 5 years (mean age, 23.3 years) with a pathologic diagnosis of micro-MTC without nodal involvement were enrolled. All patients underwent TT with thymectomy and central compartment lymph node dissection. During surgery, parathyroid tissue removal occurred in 14 patients. No major postoperative complications nor persistent hypoparathyroidism was observed. After a mean follow-up of 16.6 years, no patient developed primary hyperparathyroidism or disease recurrence. Even if TT is recommended before the age of 5, when MEN 2A diagnosis is performed after this age in micro-MTC without nodal involvement, TT with thymectomy and central compartment lymphadenectomy can provide good oncologic and functional results.
authors.affiliation: Department of Surgery and Translational Medicine, Surgical Unit, Medical School, University of Florence, Florence, Italy fmed@libero.it.
authors.forename: Francesco
authors.initials: F
authors.lastname: Tonelli
authors.affiliation: Department of Surgery and Translational Medicine, Surgical Unit, Medical School, University of Florence, Florence, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Giudici
authors.affiliation: Department of Surgery and Translational Medicine, Surgical Unit, Medical School, University of Florence, Florence, Italy.
authors.forename: Tommaso
authors.initials: T
authors.lastname: Marcucci
authors.affiliation: Department of Surgery and Translational Medicine, Surgical Unit, Medical School, University of Florence, Florence, Italy.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Cavalli
authors.affiliation: Department of Surgery and Translational Medicine, Surgical Unit, Medical School, University of Florence, Florence, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Spini
authors.affiliation: Department of Surgery and Translational Medicine, Medical School, and Regional Centre for Hereditary Endocrine Tumors, University of Florence, Florence, Italy.
authors.forename: Riccardo Gionata
authors.initials: RG
authors.lastname: Gheri
authors.affiliation: Department of Surgery and Translational Medicine, Medical School, and Regional Centre for Hereditary Endocrine Tumors, University of Florence, Florence, Italy.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Brandi
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D018278:Carcinoma, Neuroendocrine / Q000150:complications* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008197:Lymph Node Excision; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000150:complications* / Q000601:surgery*; D013934:Thymectomy; D013964:Thyroid Neoplasms / Q000150:complications* / Q000601:surgery*; D013965:Thyroidectomy
keywords: MEN 2A; N0; microscopic MTC; parathyroidectomy; thyroidectomy