Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1.
BACKGROUND
Hyperparathyroidism in patients with multiple endocrine neoplasia type 1 (MEN1) is characterized by multiglandular disease and a propensity for recurrence after parathyroidectomy (PTx). This study analyzes outcomes of a cohort of MEN1 patients undergoing initial PTx at one institution.


METHODS
Between April 1960 and September 2002, 92 patients with MEN1 underwent initial PTx. Outcomes were analyzed based on extent of parathyroid resection.


RESULTS
Fourteen percent had 2.5 or fewer glands resected, 69% had subtotal PTx, and 17% had total PTx (88% with immediate autotransplantation). The initial surgical cure rate was 98%. Excluding 6 patients lost to follow-up, 33% have developed recurrent hyperparathyroidism (in 46% after < or =2.5 PTx, in 33% after subtotal, and in 23% after total PTx). Median recurrence-free survival was not statistically significantly different between subtotal versus total PTx, but it was longer for subtotal and total PTx compared with lesser resection (16.5 vs 7.0 years, respectively, P=.03). The incidence of severe hypoparathyroidism was 46% after total versus 26% after subtotal PTx.


CONCLUSIONS
Subtotal and total PTx result in durable control of MEN1-associated hyperparathyroidism and have longer recurrence-free intervals compared with lesser resection. The high incidence of severe hypoparathyroidism after total PTx suggests that subtotal PTx is the initial operation of choice in this setting.
Elaraj
Dina M
DM
Surgery Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA.
Skarulis
Monica C
MC
Libutti
Steven K
SK
Norton
Jeffrey A
JA
Bartlett
David L
DL
Pingpank
James F
JF
Gibril
Fathia
F
Weinstein
Lee S
LS
Jensen
Robert T
RT
Marx
Stephen J
SJ
Alexander
H Richard
HR
D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000209:etiology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000601:surgery*; D016105:Parathyroidectomy / Q000379:methods*; D012008:Recurrence; D016019:Survival Analysis; D016896:Treatment Outcome