Back to Search
Start Over
Long-Term Natural Course of Small Nonfunctional Pancreatic Neuroendocrine Tumors in MEN1-Results From the Dutch MEN1 Study Group.
- Source :
-
The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2017 Oct 01; Vol. 102 (10), pp. 3795-3805. - Publication Year :
- 2017
-
Abstract
- Background: Pancreatic neuroendocrine tumors (pNETs) are highly prevalent in patients with multiple endocrine neoplasia type 1 (MEN1), and metastatic disease is an important cause of MEN1-related mortality. Especially small nonfunctional (NF) pNETs pose a challenge to the treating physician and more information is needed regarding their natural course. We assessed long-term natural history of small NF-pNETs and its modifiers in the Dutch MEN1 population.<br />Patients and Methods: Retrospective longitudinal observational cohort study of patients with small (<2 cm) NF-pNETs from the Dutch national MEN1 database, which includes >90% of the Dutch MEN1 population. Modifiers of long-term natural course were analyzed using linear mixed-models analysis.<br />Results: Growth rate of the 115 included small NF-pNETs from 99 patients was slow (0.4 mm/y; 95% confidence interval, 0.15 to 0.59). Seventy percent of the tumors was stable and a subgroup of 30% of the tumors was growing (1.6 mm/y; 95% confidence interval, 1.1 to 2.0). No differences in clinical characteristics were identified between growing and stable tumors. Within the subgroup of growing tumors, germline missense mutations were significantly associated with accelerated growth compared with nonsense and frameshift mutations.<br />Conclusion: The majority of small NF-pNETs are stable at long-term follow-up, irrespective of the underlying MEN1 genotype. A subgroup of tumors is slowly growing but cannot be identified on clinical grounds. In this subgroup, tumors with missense mutations exhibited faster growth. Additional events appear necessary for pNETs to progress. Future studies should be aimed at identifying these molecular driving events, which could be used as potential biomarkers.<br /> (Copyright © 2017 Endocrine Society)
- Subjects :
- Adult
Disease Progression
Female
Humans
Longitudinal Studies
Male
Middle Aged
Multiple Endocrine Neoplasia Type 1 complications
Multiple Endocrine Neoplasia Type 1 diagnosis
Neuroendocrine Tumors complications
Neuroendocrine Tumors diagnosis
Pancreatic Neoplasms complications
Pancreatic Neoplasms diagnosis
Prognosis
Retrospective Studies
Time Factors
Young Adult
Multiple Endocrine Neoplasia Type 1 pathology
Neuroendocrine Tumors pathology
Pancreatic Neoplasms pathology
Tumor Burden
Subjects
Details
- Language :
- English
- ISSN :
- 1945-7197
- Volume :
- 102
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- The Journal of clinical endocrinology and metabolism
- Publication Type :
- Academic Journal
- Accession number :
- 28938468
- Full Text :
- https://doi.org/10.1210/jc.2017-00372