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

Stuart D Wilson; Elizabeth A Krzywda; Yong-ran Zhu; Tina W F Yen; Tracy S Wang; Sonia L Sugg; Sam G Pappas

doi:10.1016/j.surg.2008.06.015

Back

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

Journal article

Peer reviewed

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

Stuart D Wilson, Elizabeth A Krzywda, Yong-ran Zhu, Tina W F Yen, Tracy S Wang, Sonia L Sugg and Sam G Pappas

Surgery, Vol.144(4), pp.695-702

10/2008

DOI: 10.1016/j.surg.2008.06.015

PMID: 18847656

View Online

Abstract

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. 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. 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. 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.

Registries

Pituitary Neoplasms - surgery

Age Distribution

Follow-Up Studies

Humans

Middle Aged

Male

Multiple Endocrine Neoplasia Type 1 - mortality

Multiple Endocrine Neoplasia Type 1 - genetics

Cause of Death

Parathyroid Neoplasms - mortality

Time Factors

Aged, 80 and over

Adult

Female

Pancreatic Neoplasms - mortality

Retrospective Studies

Genetic Predisposition to Disease - epidemiology

Surgical Procedures, Operative - methods

Risk Assessment

Pituitary Neoplasms - genetics

Surgical Procedures, Operative - mortality

Pancreatic Neoplasms - surgery

Pituitary Neoplasms - mortality

Pancreatic Neoplasms - genetics

Treatment Outcome

Parathyroid Neoplasms - surgery

Parathyroid Neoplasms - genetics

Syndrome

Pedigree

Survival Analysis

Sex Distribution

Aged

Multiple Endocrine Neoplasia Type 1 - surgery

Cohort Studies

Details

Title: Subtitle: The influence of surgery in MEN-1 syndrome: observations over 150 years
Creators: Stuart D Wilson - Endocrine Surgery Study Group, Department of Surgery, Medical College of Wisconsin and Froedtert Hospital, Milwaukee, WI 53226, USA. rmccoy@mcw.edu
Elizabeth A Krzywda
Yong-ran Zhu
Tina W F Yen
Tracy S Wang
Sonia L Sugg
Sam G Pappas
Resource Type: Journal article
Publication Details: Surgery, Vol.144(4), pp.695-702
Publisher: United States
DOI: 10.1016/j.surg.2008.06.015
PMID: 18847656
ISSN: 0039-6060
eISSN: 1532-7361
Language: English
Date published: 10/2008
Academic Unit: Surgery
Record Identifier: 9984051526502771

Metrics

32 Record Views

13 Times Cited - Web of Science