PMID- 22080849
OWN - NLM
STAT- MEDLINE
DCOM- 20130425
LR  - 20220410
IS  - 1720-8386 (Electronic)
IS  - 0391-4097 (Linking)
VI  - 35
IP  - 9
DP  - 2012 Oct
TI  - Natural history of gastro-entero-pancreatic and thoracic neuroendocrine tumors. 
      Data from a large prospective and retrospective Italian epidemiological study: 
      the NET management study.
PG  - 817-23
LID - 10.3275/8102 [doi]
AB  - BACKGROUND: The few epidemiological data available in literature on 
      neuroendocrine tumors (NET) are mainly based on Registry databases, missing 
      therefore details on their clinical and natural history. AIM: To investigate 
      epidemiology, clinical presentation, and natural history of NET. DESIGN AND 
      SETTING: A large national retrospective survey was conducted in 13 Italian 
      referral centers. Among 1203 NET, 820 originating in the thorax (T-NET), in the 
      gastro-enteropancreatic tract (GEP-NET) or metastatic NET of unknown primary 
      origin (U-NET) were enrolled in the study. RESULTS: 93% had a sporadic and 7% a 
      multiple endocrine neoplasia type 1 (MEN1)-associated tumor; 63% were GEP-NET, 
      33% T-NET, 4% U-NET. Pancreas and lung were the commonest primary sites. Poorly 
      differentiated carcinomas were <10%, all sporadic. The incidence of NET had a 
      linear increase from 1990 to 2007 in all the centers. The mean age at diagnosis 
      was 60.0 +/- 16.4 yr, significantly anticipated in MEN1 patients (47.7 +/- 16.5 yr). 
      Association with cigarette smoking and other non-NET cancer were more prevalent 
      than in the general Italian population. The first symptoms of the disease were 
      related to tumor burden in 46%, endocrine syndrome in 23%, while the diagnosis 
      was fortuity in 29%. Insulin (37%) and serotonin (35%) were the most common 
      hormonal hypersecretions. An advanced tumor stage was found in 42%, more 
      frequently in the gut and thymus. No differences in the overall survival was 
      observed between T-NET and GEP-NET and between sporadic and MEN1-associated 
      tumors at 10 yr from diagnosis, while survival probability was dramatically 
      reduced in U-NET. CONCLUSIONS: The data obtained from this study furnish relevant 
      information on epidemiology, natural history, and clinico-pathological features 
      of NET, not available from the few published Register studies.
FAU - Faggiano, A
AU  - Faggiano A
AD  - Department of Molecular and Clinical Endocrinology and Oncology, Section of 
      Endocrinology, University of Naples "Federico II", Italy.
FAU - Ferolla, P
AU  - Ferolla P
FAU - Grimaldi, F
AU  - Grimaldi F
FAU - Campana, D
AU  - Campana D
FAU - Manzoni, M
AU  - Manzoni M
FAU - Davi, M V
AU  - Davi MV
FAU - Bianchi, A
AU  - Bianchi A
FAU - Valcavi, R
AU  - Valcavi R
FAU - Papini, E
AU  - Papini E
FAU - Giuffrida, D
AU  - Giuffrida D
FAU - Ferone, D
AU  - Ferone D
FAU - Fanciulli, G
AU  - Fanciulli G
FAU - Arnaldi, G
AU  - Arnaldi G
FAU - Franchi, G M
AU  - Franchi GM
FAU - Francia, G
AU  - Francia G
FAU - Fasola, G
AU  - Fasola G
FAU - Crino, L
AU  - Crino L
FAU - Pontecorvi, A
AU  - Pontecorvi A
FAU - Tomassetti, P
AU  - Tomassetti P
FAU - Colao, A
AU  - Colao A
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Research Support, Non-U.S. Gov't
DEP - 20111109
PL  - Italy
TA  - J Endocrinol Invest
JT  - Journal of endocrinological investigation
JID - 7806594
RN  - Gastro-enteropancreatic neuroendocrine tumor
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Child, Preschool
MH  - Disease Management
MH  - Female
MH  - Humans
MH  - Infant
MH  - Intestinal Neoplasms/*epidemiology/mortality/therapy
MH  - Italy/epidemiology
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*epidemiology/mortality/therapy
MH  - Neuroendocrine Tumors/*epidemiology/mortality/therapy
MH  - Pancreatic Neoplasms/*epidemiology/mortality/therapy
MH  - Prevalence
MH  - Prognosis
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - Stomach Neoplasms/*epidemiology/mortality/therapy
MH  - Survival Rate
MH  - Thoracic Neoplasms/*epidemiology/mortality/therapy
MH  - Young Adult
EDAT- 2011/11/15 06:00
MHDA- 2013/04/26 06:00
CRDT- 2011/11/15 06:00
PHST- 2011/11/15 06:00 [entrez]
PHST- 2011/11/15 06:00 [pubmed]
PHST- 2013/04/26 06:00 [medline]
AID - 8102 [pii]
AID - 10.3275/8102 [doi]
PST - ppublish
SO  - J Endocrinol Invest. 2012 Oct;35(9):817-23. doi: 10.3275/8102. Epub 2011 Nov 9.