PMID- 28429092
OWN - NLM
STAT- MEDLINE
DCOM- 20180321
LR  - 20181202
IS  - 1432-2323 (Electronic)
IS  - 0364-2313 (Linking)
VI  - 41
IP  - 9
DP  - 2017 Sep
TI  - Natural History of MEN1 GEP-NET: Single-Center Experience After a Long Follow-Up.
PG  - 2312-2323
LID - 10.1007/s00268-017-4019-2 [doi]
AB  - BACKGROUND: The multiple endocrine neoplasia type 1 syndrome (MEN1) natural 
      history is poorly evaluated, and few single-institution experiences about 
      hereditary gastroenteropancreatic neuroendocrine tumors (GEP-NET) are reported. 
      Our purpose is to analyze the role of GEP-NET in MEN1-related death, as well as 
      the behavior of these lesions during follow-up. METHODS: The study population 
      consists of 77 patients diagnosed with MEN1 GEP-NET, regularly followed up since 
      1990. Extensive clinical data were prospectively recorded. Statistical analysis 
      was performed both on the whole population of 77 patients and on two subgroups 
      including patients who, during the long lasting study period, underwent GEP-NET 
      surgery (50 pts) and who did not (27 pts), respectively. RESULTS: Twenty-five 
      males (32.5%) and 52 females (67.5%) were enrolled. Sixty-four patients had MEN1 
      family history (83.1%), and genetic mutation was detected in 67 cases (87%). The 
      mean age at GEP-NET diagnosis was 41.4 years (SD = 13.6); 16 patients (20.8%) had 
      GEP-NET diagnosed before age 30 and 12 cases (15.6%) before 1996. The mean 
      interval time between MEN1 diagnosis and GEP-NET detection was 5.7 years (range 
      -11/37; SD = 8.1 years). Overall, the mean follow-up time from MEN1 diagnosis was 
      15.8 years (SD = 9.7 years) and from GEP-NET diagnosis was 9.6 years 
      (SD = 6.9 years). Gastrinoma was the most frequent functioning GEP-NET and 
      pancreatoduodenectomy the most adopted surgery. GEP-NET progression affected 12 
      patients within the non-surgical group, while 18 subjects developed progression 
      after surgery. CONCLUSIONS: Our single-center data provide information on 
      epidemiologic, clinical and pathological features of GEP-NET in MEN1 making 
      possible to clarify their natural history.
FAU - Giudici, Francesco
AU  - Giudici F
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy. francesco.giudici1@gmail.com.
FAU - Cavalli, Tiziana
AU  - Cavalli T
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy.
FAU - Giusti, Francesca
AU  - Giusti F
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy.
FAU - Gronchi, Giorgio
AU  - Gronchi G
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy.
FAU - Batignani, Giacomo
AU  - Batignani G
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy.
FAU - Tonelli, Francesco
AU  - Tonelli F
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy.
FAU - Brandi, Maria Luisa
AU  - Brandi ML
AD  - Department of Surgery and Translational Medicine, University of Florence, Largo 
      Brambilla 3, 50139, Florence, Italy.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
RN  - Gastro-enteropancreatic neuroendocrine tumor
SB  - IM
MH  - Adult
MH  - Disease Progression
MH  - Female
MH  - Follow-Up Studies
MH  - Gastrinoma/*diagnosis/genetics/mortality/surgery
MH  - Humans
MH  - *Intestinal Neoplasms/diagnosis/genetics/mortality/surgery
MH  - Male
MH  - Middle Aged
MH  - *Multiple Endocrine Neoplasia Type 1/diagnosis/genetics/mortality/surgery
MH  - Mutation
MH  - *Neuroendocrine Tumors/diagnosis/genetics/mortality/surgery
MH  - *Pancreatic Neoplasms/diagnosis/genetics/mortality/surgery
MH  - Pancreaticoduodenectomy
MH  - *Stomach Neoplasms/diagnosis/genetics/mortality/surgery
MH  - Survival Rate
MH  - Time Factors
MH  - Young Adult
EDAT- 2017/04/22 06:00
MHDA- 2018/03/22 06:00
CRDT- 2017/04/22 06:00
PHST- 2017/04/22 06:00 [pubmed]
PHST- 2018/03/22 06:00 [medline]
PHST- 2017/04/22 06:00 [entrez]
AID - 10.1007/s00268-017-4019-2 [pii]
AID - 10.1007/s00268-017-4019-2 [doi]
PST - ppublish
SO  - World J Surg. 2017 Sep;41(9):2312-2323. doi: 10.1007/s00268-017-4019-2.