Prophylactic thyroidectomy in MEN 2A syndrome

Franco Mattavelli; Ettore Seregni; Paola Collini; Barbara Pasini; Antonella Aiello; Carmen Barbaccia; Gabriella Bimbi; Stefano Riccio; Sergio Santamaria

doi:10.1177/030089160308900521

Prophylactic thyroidectomy in MEN 2A syndrome

Tumori. 2003 Sep-Oct;89(5):553-5. doi: 10.1177/030089160308900521.

Authors

Franco Mattavelli¹, Ettore Seregni, Paola Collini, Barbara Pasini, Antonella Aiello, Carmen Barbaccia, Gabriella Bimbi, Stefano Riccio, Sergio Santamaria

Affiliation

¹ Department of Head and Neck Surgery, National Cancer Institute, Milan, Italy.

PMID: 14870785
DOI: 10.1177/030089160308900521

Abstract

Genetic testing is the appropriate procedure in MEN 2A syndrome for the early diagnosis of medullary carcinoma even at a preclinical stage. Prophylactic total thyroidectomy represents the standard preventive and therapeutic surgical approach in the treatment of medullary thyroid carcinoma in MEN 2A syndrome. Our experience has confirmed the presence of CCH and medullary thyroid carcinoma even in clinically negative patients, in agreement with reports in the literature.

MeSH terms

Adult
Carcinoma, Medullary / genetics
Carcinoma, Medullary / prevention & control*
Carcinoma, Medullary / surgery*
Female
Genetic Counseling
Genetic Predisposition to Disease
Heterozygote
Humans
Male
Middle Aged
Multiple Endocrine Neoplasia Type 2a / diagnosis
Multiple Endocrine Neoplasia Type 2a / genetics
Multiple Endocrine Neoplasia Type 2a / prevention & control*
Mutation
Oncogene Proteins / genetics*
Pedigree
Primary Prevention / methods
Proto-Oncogene Proteins c-ret
Receptor Protein-Tyrosine Kinases / genetics*
Syndrome
Thyroid Neoplasms / genetics
Thyroid Neoplasms / prevention & control*
Thyroid Neoplasms / surgery*
Thyroidectomy*

Substances

Oncogene Proteins
Proto-Oncogene Proteins c-ret
RET protein, human
Receptor Protein-Tyrosine Kinases