The influence of surgery in MEN-1 syndrome: Observations over 150 years

• Published in: Surgery Vol. 144; no. 4; pp. 695 - 702
• Main Authors: Wilson, Stuart D., MD, Krzywda, Elizabeth A., NP, Zhu, Yong-ran, MD, Yen, Tina W.F., MD, MS, Wang, Tracy S., MD, MPH, Sugg, Sonia L., MD, Pappas, Sam G., MD
• Format: Journal Article
• Language: English
• Published: United States Mosby, Inc 01.10.2008
• Subjects: Adult; Age Distribution; Aged; Aged, 80 and over; Cause of Death; Cohort Studies; Female; Follow-Up Studies; Genetic Predisposition to Disease - epidemiology; Humans; Male; Middle Aged; Multiple Endocrine Neoplasia Type 1 - genetics; Multiple Endocrine Neoplasia Type 1 - mortality; Multiple Endocrine Neoplasia Type 1 - surgery; Pancreatic Neoplasms - genetics; Pancreatic Neoplasms - mortality; Pancreatic Neoplasms - surgery; Parathyroid Neoplasms - genetics; Parathyroid Neoplasms - mortality; Parathyroid Neoplasms - surgery; Pedigree; Pituitary Neoplasms - genetics; Pituitary Neoplasms - mortality; Pituitary Neoplasms - surgery; Registries; Retrospective Studies; Risk Assessment; Sex Distribution; Surgery; Surgical Procedures, Operative - methods; Surgical Procedures, Operative - mortality; Survival Analysis; Syndrome; Time Factors; Treatment Outcome
• ISSN: 0039-6060; 1532-7361
• DOI: 10.1016/j.surg.2008.06.015

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.