We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours.
- Authors
Donegan, D.; Singh Ospina, N.; Rodriguez‐Gutierrez, R.; Al‐Hilli, Z.; Thompson, G.B.; Clarke, B.L.; Young, W.F.
- Abstract
Background In patients with multiple endocrine neoplasia type 1 ( MEN-1), pancreaticoduodenal ( PD) neuroendocrine tumours ( NETs) are associated with early mortality, yet the best treatment strategy remains uncertain. Aim To assess patient important outcomes (mortality and metastasis) of PD- NETs and predictors of outcomes in patients with MEN-1. Methods Retrospective cohort of patients with MEN-1 who attended the Mayo Clinic, Rochester, MN from 1997 to 2014. Results We identified 287 patients with MEN-1; 199 (69%) patients had 217 PD- NETs. Among those with a PD- NETs, 129 (65%) had surgery of which 90 (70%) had their primary surgery performed at Mayo Clinic. The median postoperative follow-up was 8 years during which 13 (14%) patients died. The mean (±standard deviation) age of death was 51 (±9) years. Tumour size, metastasis at surgery or tumour type were not predictive of mortality, but for every year older at surgery, the odds of metastasis increased by 6%. Surgery was not performed in 70 (35%) patients. Among those who were observed/medically managed without known metastatic disease, mean tumour growth was 0·02 cm/year (range, −0·13-0·4 cm/year). Four patients (7%) died at a median age of 77 (range, 51-89) years. Conclusion PD- NETs are common in patients with MEN-1 and are associated with early mortality even after surgical intervention. Active surveillance is a viable option in nonaggressive PD- NETs, although definitive factors identifying such patients are lacking. Therefore, counselling regarding risks and benefits of current treatment options remains integral to the care of patients with MEN-1.
- Subjects
ENDOCRINE gland cancer; ENDOCRINE system; ENDOCRINE gland tumors; MULTIPLE tumors; CHROMAFFIN cell tumors; DISEASES
- Publication
Clinical Endocrinology, 2017, Vol 86, Issue 2, p199
- ISSN
0300-0664
- Publication type
Article
- DOI
10.1111/cen.13264