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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Penetrance of Functioning and Nonfunctioning Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1 in the Second Decade of Life

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Goncalves, Tatiana D. [1, 2] ; Toledo, Rodrigo A. [2, 1] ; Sekiya, Tomoko [2, 1] ; Matuguma, Sergio E. [3] ; Maluf Filho, Fauze [3] ; Rocha, Manoel S. [4] ; Siqueira, Sheila A. C. [5] ; Glezer, Andrea [1] ; Bronstein, Marcelo D. [1] ; Pereira, Maria A. A. [1] ; Jureidini, Ricardo [6] ; Bacchella, Telesforo [7, 6] ; Machado, Marcel C. C. [7] ; Toledo, Sergio P. A. [1, 2] ; Lourenco, Jr., Delmar M. [1, 2, 8]
Total Authors: 15
[1] Univ Sao Paulo, Sch Med, Hosp Clin, Div Endocrinol, BR-01246903 Sao Paulo - Brazil
[2] Univ Sao Paulo, Sch Med, Hosp Clin, Endocrine Genet Unit LIM25, BR-01246903 Sao Paulo - Brazil
[3] Univ Sao Paulo, Sch Med, Hosp Clin, Endoscopy Div, BR-01246903 Sao Paulo - Brazil
[4] Univ Sao Paulo, Sch Med, Hosp Clin, Div Radiol, BR-01246903 Sao Paulo - Brazil
[5] Univ Sao Paulo, Sch Med, Hosp Clin, Div Pathol, BR-01246903 Sao Paulo - Brazil
[6] Canc Inst Sao Paulo, Div Surg, Dept Gastroenterol, BR-01246000 Sao Paulo - Brazil
[7] Univ Sao Paulo, Sch Med, Hosp Clin, Div Surg, Dept Gastroenterol, BR-01246903 Sao Paulo - Brazil
[8] Canc Inst Sao Paulo, Endocrine Oncol Div, BR-01246000 Sao Paulo - Brazil
Total Affiliations: 8
Document type: Journal article
Web of Science Citations: 28

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. Apotential 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 was42%(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. (AU)

FAPESP's process: 09/15386-6 - Analysis of the CDKN1A, CDKN1B, CDKN2B and CDKN2C genes in multiple endocrine neoplasias type 1 and 2.
Grantee:Rodrigo de Almeida Toledo
Support Opportunities: Scholarships in Brazil - Post-Doctorate
FAPESP's process: 09/11942-1 - Analysis of CDKN1B/p27kip1 gene in patients with Multiple Endocrine Neoplasia Type 2
Grantee:Tomoko Sekiya
Support Opportunities: Scholarships in Brazil - Doctorate (Direct)