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.
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 MEN1patients. 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 MEN1patients in a tertiary academic reference center. PATIENTS: Nineteen MEN1patients (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 MEN1patients. 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.
Authors: James R Howe; Nipun B Merchant; Claudius Conrad; Xavier M Keutgen; Julie Hallet; Jeffrey A Drebin; Rebecca M Minter; Terry C Lairmore; Jennifer F Tseng; Herbert J Zeh; Steven K Libutti; Gagandeep Singh; Jeffrey E Lee; Thomas A Hope; Michelle K Kim; Yusuf Menda; Thorvardur R Halfdanarson; Jennifer A Chan; Rodney F Pommier Journal: Pancreas Date: 2020-01 Impact factor: 3.327
Authors: Jonathan D Wasserman; Gail E Tomlinson; Harriet Druker; Junne Kamihara; Wendy K Kohlmann; Christian P Kratz; Katherine L Nathanson; Kristian W Pajtler; Andreu Parareda; Surya P Rednam; Lisa J States; Anita Villani; Michael F Walsh; Kristin Zelley; Joshua D Schiffman Journal: Clin Cancer Res Date: 2017-07-01 Impact factor: 12.531
Authors: Jerena Manoharan; Friedhelm Raue; Caroline L Lopez; Max B Albers; Carmen Bollmann; Volker Fendrich; Emily P Slater; Detlef K Bartsch Journal: World J Surg Date: 2017-08 Impact factor: 3.352
Authors: M Falconi; B Eriksson; G Kaltsas; D K Bartsch; J Capdevila; M Caplin; B Kos-Kudla; D Kwekkeboom; G Rindi; G Klöppel; N Reed; R Kianmanesh; R T Jensen Journal: Neuroendocrinology Date: 2016-01-05 Impact factor: 4.914
Authors: Wei Qiu; Ioannis Christakis; Angelica Silva; Roland L Bassett; Liyun Cao; Qing H Meng; Elizabeth Gardner Grubbs; Hua Zhao; James C Yao; Jeffrey E Lee; Nancy D Perrier Journal: Clin Endocrinol (Oxf) Date: 2016-06-30 Impact factor: 3.478