A Standardized Meal Stimulation Test of the Endocrine Pancreas for Early Detection of Pancreatic Endocrine Tumors in Multiple Endocrine Neoplasia Type 1 Syndrome: Five Years Experience*

Abstract

Forty-nine members of 6 families with multiple endocrine neoplasia type 1 (MEN 1) were investigated with a standardized meal stimulation test to detect the presence of pancreatic endocrine tumors. Fifteen age-matched subjects and 4 patients with primary hyperparathyroidism also were studied. Serum pancreatic polypeptide (PP), gastrin, and insulin as well as plasma glucagon and somatostatin concentrations were determined before and during the test meal. Patients with demonstrable pancreatic endocrine tumors had significantly increased mean basal and peak serum PP (P less than 0.001) and gastrin (P less than 0.001) responses to the meal compared with healthy family members and normal subjects. Seven of 12 MEN 1 patients with parathyroid and pituitary disease but no demonstrable pancreatic endocrine tumors had exaggerated PP and/or gastrin responses to the meal; 4 of them developed pancreatic endocrine tumors, detected by abdominal computerized tomography, 0.5-4 yr later. None of the healthy members of the MEN 1 families or the patients with primary hyperparathyroidism had responses different from those of the normal subjects. Our experience with the meal stimulation test indicates that an elevated basal or exaggerated serum PP and/or gastrin response is an earlier sign of pancreatic involvement in the MEN 1 trait than is abdominal computerized tomography.

Surgical Treatment of Endocrine Pancreatic Lesions in Men-1

“…Multiple hormones have been identified in 40-70% of detected tumors (6, 7). Kloppel et al (7) found a high prevalence of pancreatic polypeptide (PP)-immunoreactive lesions, corroborating the value of basal or stimulated PP-measurements for screening in MEN-I (3,4,9). Although larger tumors have frequently shown PP-immunoreactivity, no specific symptoms have been related to this hormone excess (10, 1 I).…”

Neuroendocrine Tumors of the Gastro-Entero-Pancreatic System: The Role of Early Diagnosis, Genetic Testing and Preventive Surgery

“…Thus, annual screening with sensitive tumor markers (chromogranin A or pancreatic polypeptide) is highly recommended. A meal stimulation test with determination of PP has been proposed for early diagnosis with an exaggerated increase of PP indicative of a pancreatic tumor [104]. For intensive investigation to start, two independent tumor markers should be elevated.…”

The molecular genetics of the multiple endocrine neoplasia syndromes

“…Pancreatic involvement in asymptomatic individuals has previously been detected by estimating the fasting plasma concentrations of gastrin and PP. However, one recent study has reported that a stimulatory meal test is a better method for detecting pancreatic disease in individuals who have no pancreatic tumours demonstrable by computerized tomography (Skogseid et al, 1987). An exaggerated increase in serum gastrin and/or PP proved to be a reliable early indicator for the development of pancreatic tumours in these individuals.…”