Document Detail

Penetrance of functioning and non-functioning pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 in the second decade of life.
MedLine Citation:
PMID:  24178797     Owner:  NLM     Status:  Publisher    
Context:Data are scarce on penetrance of multiple endocrine neoplasia type 1 (MEN1)-related non-functioning pancreatic neuroendocrine tumors (NF-PETs) and insulinomas in young MEN1 patients. A potential positive correlation between tumor size and malignancy (2-3cm, 18%; >3cm, 43%) has greatly influenced the management of MEN1 adults with NF-PETs.Objective: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 (tertiary academic reference center).Patients:Nineteen MEN1 patients (aged 12-20y; 16 patients aged 15-20y and 3 patients aged 12-14y) were screened for NF-PETs, insulinomas and gastrinomas.Methods:MRI/CT and endoscopic US (EUS) were performed on 10 MEN1 carriers, MRI/CT 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% (8/19). All eight PET patients had NF-PETs, and half of those tumors were multicentric. One-fifth of the screened patients (21%; 4/19) harbored at least one large tumor (>2.0cm). 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 15-20y 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 (>2.0cm; T2N0M0) that were classified as grade 1 neuroendocrine tumors (Ki-67<2%).Conclusions:Our data demonstrated high penetrance of NF-PETs in 15-20-y-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.
Tatiana D Gonçalves; Rodrigo A Toledo; Tomoko Sekiya; Sergio E Matuguma; Fauze Maluf Filho; Manoel S Rocha; Sheila A C Siqueira; Andrea Glezer; Marcelo D Bronstein; Maria A A Pereira Ricardo Jureidini; Telesforo Bacchella; Marcel C C Machado; Sergio P A Toledo; Delmar M Lourenço
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-10-31
Journal Detail:
Title:  The Journal of clinical endocrinology and metabolism     Volume:  -     ISSN:  1945-7197     ISO Abbreviation:  J. Clin. Endocrinol. Metab.     Publication Date:  2013 Oct 
Date Detail:
Created Date:  2013-11-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375362     Medline TA:  J Clin Endocrinol Metab     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Endocrine Genetics Unit (LIM-25) (T.D.G., R.A.T.*, T.S., S.P.A.T., D.M.L.J.), 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.J.), Endoscopy Division (S.E.M., F.M.F.), Radiology Division (M.S.R.), Pathology Division (S.A.C.S.), Department of Gastroenterology, Surgical Division (T.B., M.C.C.M.), Hospital das Clínicas, University of São Paulo School of Medicine; Department of Gastroenterology, Surgical Division (T.B., R.J.) and Endocrine Oncology Division, Cancer Institute of São Paulo (D.M.L.J.), São Paulo, Brazil.; * R.A.T is now at Division of Hematology and Medical Oncology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Dr, MC 7880, San Antonio, TX 78229-3900, USA.
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