PMID- 27770475
OWN - NLM
STAT- MEDLINE
DCOM- 20170922
LR  - 20220331
IS  - 1365-2265 (Electronic)
IS  - 0300-0664 (Linking)
VI  - 86
IP  - 2
DP  - 2017 Feb
TI  - Long-term outcomes in patients with multiple endocrine neoplasia type 1 and 
      pancreaticoduodenal neuroendocrine tumours.
PG  - 199-206
LID - 10.1111/cen.13264 [doi]
AB  - 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.
CI  - (c) 2016 John Wiley & Sons Ltd.
FAU - Donegan, D
AU  - Donegan D
AD  - Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, 
      Rochester, MN, USA.
FAU - Singh Ospina, N
AU  - Singh Ospina N
AD  - Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, 
      Rochester, MN, USA.
AD  - Knowledge and Evaluation Research Unit in Endocrinology (KER-Endo), Division of 
      Endocrinology, Diabetes, Metabolism and Nutrition, Department of Medicine, Mayo 
      Clinic, Rochester, MN, USA.
FAU - Rodriguez-Gutierrez, R
AU  - Rodriguez-Gutierrez R
AD  - Knowledge and Evaluation Research Unit in Endocrinology (KER-Endo), Division of 
      Endocrinology, Diabetes, Metabolism and Nutrition, Department of Medicine, Mayo 
      Clinic, Rochester, MN, USA.
AD  - Division of Endocrinology, Department of Internal Medicine, University Hospital 
      "Dr. Jose E. Gonzalez", Autonomous University of Nuevo Leon, Monterrey, Mexico.
FAU - Al-Hilli, Z
AU  - Al-Hilli Z
AD  - Division of Surgery, Mayo Clinic, Rochester, MN, USA.
FAU - Thompson, G B
AU  - Thompson GB
AD  - Division of Surgery, Mayo Clinic, Rochester, MN, USA.
FAU - Clarke, B L
AU  - Clarke BL
AD  - Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, 
      Rochester, MN, USA.
FAU - Young, W F Jr
AU  - Young WF Jr
AD  - Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, 
      Rochester, MN, USA.
LA  - eng
PT  - Journal Article
DEP - 20161117
PL  - England
TA  - Clin Endocrinol (Oxf)
JT  - Clinical endocrinology
JID - 0346653
SB  - IM
MH  - Adult
MH  - Cohort Studies
MH  - Follow-Up Studies
MH  - Humans
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*complications/mortality/pathology
MH  - Neoplasm Metastasis
MH  - Neuroendocrine Tumors/*complications/mortality/surgery
MH  - Pancreatic Neoplasms/*chemistry/mortality/surgery
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - Young Adult
EDAT- 2016/10/23 06:00
MHDA- 2017/09/25 06:00
CRDT- 2016/10/23 06:00
PHST- 2016/08/03 00:00 [received]
PHST- 2016/10/10 00:00 [revised]
PHST- 2016/10/19 00:00 [accepted]
PHST- 2016/10/23 06:00 [pubmed]
PHST- 2017/09/25 06:00 [medline]
PHST- 2016/10/23 06:00 [entrez]
AID - 10.1111/cen.13264 [doi]
PST - ppublish
SO  - Clin Endocrinol (Oxf). 2017 Feb;86(2):199-206. doi: 10.1111/cen.13264. Epub 2016 
      Nov 17.