Abstract
Background and objectivesGastrinomas are the most prevalent functioning neuroendocrine tumors (NET) in multiple endocrine neoplasia type 1 (MEN1). Guidelines suggest medical therapy in most patients, but surgery may be considered in a subgroup. Currently, factors to guide management are necessary. This population‐based cohort study assessed prognostic factors of survival in patients with MEN1‐related gastrinomas.MethodsPatients with MEN1 having gastrinomas were identified in the Dutch MEN1 database from 1990 to 2014 based on fasting serum gastrin (FSG) levels and/or pathology. Predictors of overall survival were assessed using Cox regression.ResultsSixty‐three patients with gastrinoma (16% of the MEN1 population) were identified. Five‐ and 10‐year overall survival rates were 83% and 65%, respectively. Prognostic factors associated with overall survival were initial FSG levels ≥20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7‐23.0]), pancreatic NET ≥2 cm (HR 4.5; [1.5‐13.1]), synchronous liver metastases (HR 8.9; [2.1‐36.7]), gastroduodenoscopy suspicious for gastric NETs (HR 12.7; [1.4‐115.6]), and multiple concurrent NETs (HR 5.9; [1.2‐27.7]).ConclusionLife expectancy of patients with MEN1 gastrinoma is reduced. FSG levels and pancreatic NETs ≥2 cm are prognostic factors. FSG levels might guide surveillance intensity, step‐up to additional diagnostics, or provide arguments in selecting patients who might benefit from surgery.
Highlights
Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder caused by a mutation in the MEN1 gene leading to a combination of endocrine and nonendocrine tumors.[1]
Factors significantly associated with overall survival (OS) were fasting serum gastrin (FSG) levels >20x ULN (HR, 6.16; [95% confidence interval (CI), 1.65‐23.02]), Pancreatic neuroendocrine tumor (pNET) ≥2.0 cm on conventional imaging (HR, 4.46; [1.52‐ 13.06]), synchronous liver metastases of any origin (HR, 8.86; [2.14‐ 36.7]), multiple concurrent neuroendocrine tumor (NET) (HR, 5.86; [1.24‐27.65]), and gastroduodenoscopy suspicious for gastric NET
Factors associated with decreased OS were initial FSG levels ≥20x ULN, a pNET ≥2.0 cm on conventional imaging, synchronous liver metastases, multiple concurrent NETs, and gastroduodenoscopy suspicious for gastric NET
Summary
Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder caused by a mutation in the MEN1 gene leading to a combination of endocrine and nonendocrine tumors.[1]. This population‐based cohort study assessed prognostic factors of survival in patients with MEN1‐related gastrinomas. Prognostic factors associated with overall survival were initial FSG levels ≥20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7‐23.0]), pancreatic NET ≥2 cm (HR 4.5; [1.5‐13.1]), synchronous liver metastases
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