PMID- 21550956
OWN - NLM
STAT- MEDLINE
DCOM- 20120126
LR  - 20210204
IS  - 1934-2403 (Electronic)
IS  - 1530-891X (Linking)
VI  - 17 Suppl 3
DP  - 2011 Jul-Aug
TI  - Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: 
      review of literature.
PG  - 33-40
LID - 10.4158/EP10376.RA [doi]
AB  - OBJECTIVE: To analyze the surgical approach to multiple endocrine neoplasia type 
      1 (MEN 1)-related pancreatic endocrine tumors (PETs). METHODS: We reviewed 
      selected publications and our personal experience with MEN 1-associated PETs to 
      delineate their general characteristics, current practice and controversies, 
      preoperative imaging and intraoperative assessment, and appropriate therapeutic 
      strategies including radical surgical procedures. RESULTS: The penetrance of PETs 
      in the setting of MEN 1 is similar to that of parathyroid tumors, even though 
      hyperparathyroidism is usually the first manifestation of MEN 1 syndrome. In 
      contrast with the sporadic counterparts, MEN 1-related PETs are characterized by 
      an early onset, multiplicity of lesions, variable expression of the tumors, and 
      propensity for malignant degeneration. Both the histologic type and the size of 
      these tumors correlate with malignant potential. CONCLUSION: The rationale for 
      surgical considerations for these tumors is to curtail the malignant progression 
      of the disease and to cure or aid in management of the associated biochemical 
      syndromes. A surgical procedure is often the treatment of choice for PETs in 
      patients with MEN 1. Monitoring of pancreatic peptides and use of diagnostic 
      imaging allow an early pancreatic resection, in conjunction with prevention of 
      metastatic PETs and improvement of long-term survival. Hepatic metastatic lesions 
      can be successfully treated by surgical resection.
FAU - Tonelli, Francesco
AU  - Tonelli F
AD  - Department of Clinical Physiopathology, Surgical Unit, University of Florence 
      Medical School, Viale G B Morgagni 85, Florence, Italy. f.tonelli@dfc.unifi.it
FAU - Giudici, Francesco
AU  - Giudici F
FAU - Fratini, Geri
AU  - Fratini G
FAU - Brandi, Maria Luisa
AU  - Brandi ML
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Endocr Pract
JT  - Endocrine practice : official journal of the American College of Endocrinology 
      and the American Association of Clinical Endocrinologists
JID - 9607439
SB  - IM
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/*complications
MH  - Pancreatic Neoplasms/*diagnosis/surgery
EDAT- 2011/05/10 06:00
MHDA- 2012/01/27 06:00
CRDT- 2011/05/10 06:00
PHST- 2011/05/10 06:00 [entrez]
PHST- 2011/05/10 06:00 [pubmed]
PHST- 2012/01/27 06:00 [medline]
AID - S1530-891X(20)41719-7 [pii]
AID - 10.4158/EP10376.RA [doi]
PST - ppublish
SO  - Endocr Pract. 2011 Jul-Aug;17 Suppl 3:33-40. doi: 10.4158/EP10376.RA.