PMID- 24178797
OWN - NLM
STAT- MEDLINE
DCOM- 20140312
LR  - 20140103
IS  - 1945-7197 (Electronic)
IS  - 0021-972X (Linking)
VI  - 99
IP  - 1
DP  - 2014 Jan
TI  - Penetrance of functioning and nonfunctioning pancreatic neuroendocrine tumors in 
      multiple endocrine neoplasia type 1 in the second decade of life.
PG  - E89-96
LID - 10.1210/jc.2013-1768 [doi]
AB  - CONTEXT: Data are scarce on the penetrance of multiple endocrine neoplasia type 1 
      (MEN1)-related nonfunctioning pancreatic neuroendocrine tumors (NF-PETs) and 
      insulinomas in young MEN1 patients. A potential positive correlation between 
      tumor size and malignancy (2-3 cm, 18%; >3 cm, 43%) has greatly influenced the 
      management of MEN1 adults with NF-PETs. OBJECTIVE: The aim of the study was to 
      estimate the penetrance of NF-PETs, insulinomas, and gastrinomas in young MEN1 
      carriers. DESIGN: The data were obtained from a screening program (1996-2012) 
      involving 113 MEN1 patients in a tertiary academic reference center. PATIENTS: 
      Nineteen MEN1 patients (aged 12-20 y; 16 patients aged 15-20 y and 3 patients 
      aged 12-14 y) were screened for NF-PETs, insulinomas, and gastrinomas. METHODS: 
      Magnetic resonance imaging/computed tomography and endoscopic ultrasound (EUS) 
      were performed on 10 MEN1 carriers, magnetic resonance imaging/computed 
      tomography was performed on five patients, and four other patients underwent an 
      EUS. RESULTS: The overall penetrance of PETs during the second decade of life was 
      42% (8 of 19). All eight PET patients had NF-PETs, and half of those tumors were 
      multicentric. One-fifth of the screened patients (21%; 4 of 19) harbored at least 
      one large tumor (>2.0 cm). Insulinoma was detected in two NF-PET patients (11%) 
      at the initial screening; gastrinoma was not present in any cases. Six of the 11 
      (54%) screened patients aged 15-20 years who underwent an EUS had NF-PETs. 
      Potential false-positive EUS results were excluded based on EUS-guided biopsy 
      results, the reproducibility of the NF-PET findings, or the observation of 
      increased tumor size during follow-up. Distal pancreatectomy and the nodule 
      enucleation of pancreatic head tumors were conducted on three patients with large 
      tumors (>2.0 cm; T2N0M0) that were classified as grade 1 neuroendocrine tumors 
      (Ki-67<2%). CONCLUSIONS: Our data demonstrated high penetrance of NF-PETs in 15- 
      to 20-year-old MEN1 patients. The high percentage of the patients presenting 
      consensus criteria for surgery for NF-PET alone or NF-PET/insulinoma suggests a 
      potential benefit for the periodic surveillance of these tumors in this age 
      group.
FAU - Goncalves, Tatiana D
AU  - Goncalves TD
AD  - Endocrine Genetics Unit (LIM-25) (T.D.G., R.A.T., T.S., S.P.A.T., D.M.L.), 
      Endocrinology Division (T.D.G., R.A.T., T.S., A.G., M.D.B., M.A.A.P., S.P.A.T., 
      D.M.L.), Endoscopy Division (S.E.M., F.M.F.), Radiology Division (M.S.R.), 
      Pathology Division (S.A.C.S.), and Department of Gastroenterology, Surgical 
      Division (T.B., M.C.C.M.), Hospital das Clinicas, University of Sao Paulo School 
      of Medicine, Sao Paulo 01246-903, Brazil; and Department of Gastroenterology, 
      Surgical Division (T.B., R.J.), and Endocrine Oncology Division, Cancer Institute 
      of Sao Paulo (D.M.L.), Sao Paulo 01246-000, Brazil.
FAU - Toledo, Rodrigo A
AU  - Toledo RA
FAU - Sekiya, Tomoko
AU  - Sekiya T
FAU - Matuguma, Sergio E
AU  - Matuguma SE
FAU - Maluf Filho, Fauze
AU  - Maluf Filho F
FAU - Rocha, Manoel S
AU  - Rocha MS
FAU - Siqueira, Sheila A C
AU  - Siqueira SA
FAU - Glezer, Andrea
AU  - Glezer A
FAU - Bronstein, Marcelo D
AU  - Bronstein MD
FAU - Pereira, Maria A A
AU  - Pereira MA
FAU - Jureidini, Ricardo
AU  - Jureidini R
FAU - Bacchella, Telesforo
AU  - Bacchella T
FAU - Machado, Marcel C C
AU  - Machado MC
FAU - Toledo, Sergio P A
AU  - Toledo SP
FAU - Lourenco, Delmar M Jr
AU  - Lourenco DM Jr
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20131220
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
RN  - Non functioning pancreatic endocrine tumor
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age Factors
MH  - Child
MH  - Female
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/epidemiology/*genetics/pathology
MH  - Neoplasm Invasiveness
MH  - Neuroendocrine Tumors/epidemiology/*genetics/pathology
MH  - Pancreatic Neoplasms/epidemiology/*genetics/pathology
MH  - *Penetrance
MH  - Proto-Oncogene Proteins/*genetics
MH  - Review Literature as Topic
MH  - Tumor Burden
MH  - Young Adult
EDAT- 2013/11/02 06:00
MHDA- 2014/03/13 06:00
CRDT- 2013/11/02 06:00
PHST- 2013/11/02 06:00 [entrez]
PHST- 2013/11/02 06:00 [pubmed]
PHST- 2014/03/13 06:00 [medline]
AID - jc.2013-1768 [pii]
AID - 10.1210/jc.2013-1768 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2014 Jan;99(1):E89-96. doi: 10.1210/jc.2013-1768. Epub 
      2013 Dec 20.