The influence of surgery in MEN-1 syndrome: observations over 150 years. Surgery 2008 Oct;144(4):695-701; discussion 701-2
Date
10/14/2008Pubmed ID
18847656DOI
10.1016/j.surg.2008.06.015Scopus ID
2-s2.0-51449120529 (requires institutional sign-in at Scopus site) 15 CitationsAbstract
BACKGROUND: Efficacy and timing of operative intervention in patients with multiple endocrine neoplasia type 1 (MEN-1) syndrome remains controversial. This report utilizes a novel approach to evaluate the influence of evolving operative interventions for patients with MEN-1 syndrome.
METHODS: Six generations from a large MEN-1 family pedigree were studied. The number of operations for MEN-1 related pathology was recorded according to birth eras over 150 years. Length of life was a primary outcome measurement.
RESULTS: Inheritance of the MEN-1 trait was near 50%. There were no instances of a skipped generation. Affected individuals born before 1900 died from gastrointestinal hemorrhage and without any surgical intervention. After 1900, there were increasing numbers of gastric, parathyroid, and pancreatic operations in successive eras. Death occurred >20 years earlier in MEN-1 individuals than unaffected family members in eras 1 and 2. Family members with MEN-1 lived longer in succeeding eras with increasing number of operative and pharmacologic interventions.
CONCLUSION: MEN-1 family members invariably have pathologic changes in pituitary, parathyroid, and pancreatic islets when long lived, the "all-or-none" phenomenon. Patients are not cured with operative interventions, although they may live longer and without symptoms with a good quality of life. This model may allow better comparisons with other MEN-1 patients when evaluating outcomes of new medical and operative management schemes and long-term follow-up.
Author List
Wilson SD, Krzywda EA, Zhu YR, Yen TW, Wang TS, Sugg SL, Pappas SGAuthors
Tracy S. Wang MD, MPH Professor in the Surgery department at Medical College of WisconsinTina W F Yen MD, MS Professor in the Surgery department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
AdultAge Distribution
Aged
Aged, 80 and over
Cause of Death
Cohort Studies
Female
Follow-Up Studies
Genetic Predisposition to Disease
Humans
Male
Middle Aged
Multiple Endocrine Neoplasia Type 1
Pancreatic Neoplasms
Parathyroid Neoplasms
Pedigree
Pituitary Neoplasms
Registries
Retrospective Studies
Risk Assessment
Sex Distribution
Surgical Procedures, Operative
Survival Analysis
Syndrome
Time Factors
Treatment Outcome
