Objective
Pancreatic neuroendocrine tumors (PNETs) are the major source of disease-specific mortality in multiple endocrine neoplasia type 1 (MEN1) patients. Chromogranin A (CgA), pancreatic polypeptide (PP), glucagon, and gastrin have some diagnostic value in sporadic PNETs, but there is very little evidence for their efficacy in diagnosing PNETs in MEN1 patients.
Design
We performed a retrospective chart review of the existing MEN1 database in our institution.
Patients
One hundred thirteen patients were eligible for diagnostic value analysis of tumor markers. Patients were excluded if measurement of tumor markers was missing, either 3 months prior to PNETs diagnosis (PNETs patients) or prior to abdominal imaging (non-PNETs patients).
Measurements
Clinicopathologic characteristics and of tumor marker measurements were analyzed.
Results
Of 293 confirmed MEN1 cases, 55 PNETs and 58 non-PNETs met inclusion criteria. The area under the curve (AUC) for CgA, PP, glucagon, and gastrin in MEN1 cases was 59.5%, 64.1%, 77.0%, and 75.9%, respectively. The AUC for the combination of CgA, PP, and gastrin was 59.6%. PP, but not CgA, glucagon or gastrin was significantly associated with both age and PNETs functional status (P=0.0485 and 0.0188, respectively). No markers were significantly associated with sex, PNETs tumor size, tumor number, tumor location, AJCC (American Joint Committee on Cancer) stage, presence of lymph node metastasis, lymphovascular invasion, or overall survival. CgA values were not significantly lower following PNETs resection than pre-operatively (P=0.554).
Conclusions
The value of blood markers for diagnosing PNETs in MEN1 patients is relatively low, even when used in combination.