[Hereditary thyroid cancer can be cured by prophylactic surgery].
Multiple endocrine neoplasia type 2 (MEN 2) is a rare syndrome in which the consequences for the patient and family members are considerable. Mutation analysis of the RET proto-oncogene is crucial for decision-making regarding each patient. Today, carriers of MEN 2 mutations should be offered prophylactic thyroidectomy with the potential to eliminate the risk for potentially lethal medullary thyroid carcinoma (MTC). Here, we present the first Swedish experience of such operations performed mainly on the basis of genetic analysis. Twenty patients underwent total thyroidectomy at a mean age of 13.5 (6-43) years. In all cases, either manifest MTC (n = 11) or C-cell hyperplasia was found. So far, no patient has any sign of recurrence or developmental insufficiency at 1-5 years follow-up. As the medical and ethical problems in this group of patients are substantial, and as the operations are performed in otherwise healthy children, they should be treated at centers with adequate multidisciplinary expertise and competence.
Wallin
G
G
Kirurgiska kliniken, Karolinska sjukhuset, Stockholm.
Bondesson
A G
AG
Farnebo
L O
LO
Hallengren
B
B
Hamberger
B
B
Jansson
S
S
Nilsson
O
O
Nordenskjöld
M
M
Smeds
S
S
Svensson
K A
KA
Wihlborg
O
O
Zedenius
J
J
D000293:Adolescent; D000328:Adult; D018276:Carcinoma, Medullary / Q000235:genetics* / Q000517:prevention & control / Q000601:surgery; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D008297:Male; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics* / Q000517:prevention & control / Q000601:surgery; D017410:Practice Guidelines as Topic; D000090063:Proto-Oncogene Mas; D013964:Thyroid Neoplasms / Q000235:genetics* / Q000517:prevention & control / Q000601:surgery; D013965:Thyroidectomy