PMID- 16680581
OWN - NLM
STAT- MEDLINE
DCOM- 20061019
LR  - 20220409
IS  - 0364-2313 (Print)
IS  - 0364-2313 (Linking)
VI  - 30
IP  - 5
DP  - 2006 May
TI  - Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1.
PG  - 643-53
AB  - INTRODUCTION: Pancreatic endocrine tumors (PETs) occur in at least 50% of 
      patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading 
      cause of disease-specific mortality. However, the timing and extent of surgery 
      for MEN1-related PETs is controversial owing to the indolent tumor growth seen in 
      most patients and the desire to avoid complications associated with insulin 
      dependence. To help resolve this controversy, we retrospectively analyzed the 
      clinical characteristics, surgical treatment, and clinical outcome of patients 
      with MEN1-related PETs. METHODS: All patients had histologic or radiographic 
      confirmation of a PET in the setting of MEN1. Disease progression was defined 
      radiographically as the development of new pancreatic tumors or distant 
      metastases. Progression-free survival (PFS) and overall survival (OS) were used 
      as the endpoints of this analysis. RESULTS: We identified 98 patients with MEN1, 
      55 (56%) of whom had PETs, including 27 women and 28 men with a median age of 37 
      years (range 8-69 years) at the time of diagnosis. Functioning PETs were present 
      in 35 (64%) of 55 patients, and nonfunctioning tumors were present in 20 (36%). 
      Pancreatic surgery was performed in 38 (69%) of the 55 patients; and the first 
      operation included enucleation (n = 4), total pancreatectomy (n = 3), Whipple 
      procedure (n = 4), and distal pancreatectomy (n = 27). The median size of the 
      resected tumors was 2.8 cm (range 0.6-11.0 cm). Recurrent disease developed in 
      the residual pancreas in 7 (20%) of 35 at-risk patients a median of 7.8 years 
      after the first operation, and distant metastases occurred in 5 (14 %) of 36 
      surgically treated patients without distant metastasis (2 patients had distant 
      metastases when surgery on the primary tumor was performed) at a median of 2.7 
      years following surgery. At last follow-up, 16 (29%) of 55 patients with PETs had 
      died, 12 (22%) were alive with disease, 26 (47%) were alive without evidence of 
      disease, and 1 (2%) was lost to follow-up. The median OS was 19.5 years (range 
      13-26 years) and was significantly longer for patients who had functioning PETs 
      versus those with nonfunctioning tumors (P = 0.0007), for patients who underwent 
      surgical resection of their PETs versus those who did not (P = 0.0043), and for 
      patients with localized versus metastatic PETs at the time of diagnosis (P < 
      0.0001). Multivariate analysis revealed that younger age, hormonal function, and 
      PET resection were independently associated with longer OS. CONCLUSIONS: Our data 
      suggest that early diagnosis and surgical excision of MEN1-related PETs improves 
      survival. However, translating these data into a surveillance strategy for the 
      early detection of PETs is complex owing to the potential morbidity of pancreatic 
      resection and the risk of long-term insulin dependence.
FAU - Kouvaraki, Maria A
AU  - Kouvaraki MA
AD  - Department of Surgical Oncology, The University of Texas M. D. Anderson Cancer 
      Center, Unit 444, 1400 Holcombe Boulevard, Houston, Texas 77030-1402, USA.
FAU - Shapiro, Suzanne E
AU  - Shapiro SE
FAU - Cote, Gilbert J
AU  - Cote GJ
FAU - Lee, Jeffrey E
AU  - Lee JE
FAU - Yao, James C
AU  - Yao JC
FAU - Waguespack, Steven G
AU  - Waguespack SG
FAU - Gagel, Robert F
AU  - Gagel RF
FAU - Evans, Douglas B
AU  - Evans DB
FAU - Perrier, Nancy D
AU  - Perrier ND
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Child
MH  - Digestive System Surgical Procedures
MH  - Endoscopy, Digestive System
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Multiple Endocrine Neoplasia Type 1/complications
MH  - Pancreatic Neoplasms/diagnosis/mortality/*surgery
MH  - Retrospective Studies
MH  - Survival Analysis
MH  - Treatment Outcome
EDAT- 2006/05/09 09:00
MHDA- 2006/10/20 09:00
CRDT- 2006/05/09 09:00
PHST- 2006/05/09 09:00 [pubmed]
PHST- 2006/10/20 09:00 [medline]
PHST- 2006/05/09 09:00 [entrez]
AID - 10.1007/s00268-006-0360-y [doi]
PST - ppublish
SO  - World J Surg. 2006 May;30(5):643-53. doi: 10.1007/s00268-006-0360-y.