Objective: To compare clinical characteristics of sporadic
gastrinoma and
multiple endocrine neoplasia type 1 (MEN1)-related
gastrinoma. Methods: A retrospective cohort study was conducted. Patients with clinical manifestations of
Zollinger-Ellison syndrome, pathological diagnosis as neuroendocrine
neoplasm (NEN) and complete clinical and follow-up data were enrolled. Patients with only high gastric acid secretion but without evidence of NEN, or with other concurrent non-NEN
tumors were excluded. According to the above criteria, the clinicopathological data of 52 cases of
gastrinoma diagnosed from April 2003 to December 2020 in the First Affiliated Hospital, Sun Yat-sen University, were collected. Patients who met the diagnostic criteria of
gastrinoma and met one of the following conditions were diagnosed as MEN1-related
gastrinoma: (1) the presence of pathogenic mutations in the MEN1 gene confirmed by genetic testing; (2) NENs involving two or more endocrine glands, namely, pituitary, parathyroid, thymic, pancreatic, and adrenal NENs; (3) NEN and at least one first-degree relatives diagnosed as MEN1. The remaining
gastrinomas were defined as sporadic
gastrinoma. Student's t test and chi-square test were used for statistical analysis. Clinicopathological characteristics, endoscopic findings, imaging characteristics, treatment, and prognosis of sporadic and MEN1-related
gastrinoma were compared. Results: Among 52 patients with
gastrinoma, 33 were sporadic
gastrinoma and 19 were MEN1-related
gastrinoma. The common symptoms of both sporadic and MEN1-related
gastrinomas were
diarrhea (24/33, 72.7%; 17/19, 89.5%) and
abdominal pain (19/33, 57.6%; 9/19, 47.4%). Compared with sporadic
gastrinoma, MEN1-related
gastrinoma needed longer time for diagnosis [(7.4±4.9) years vs. (3.9±5.2) years, t=-2.355, P=0.022), were more likely multiple
tumors [47.4% (9/19) vs. 15.2% (5/33), χ(2)=6.361, P=0.012], had smaller diameter [(1.7±1.0) cm vs. (3.1±1.8) cm, t=2.942, P=0.005), presented the lower
tumor grade [G1: 83.3% (15/18) vs. 39.4% (13/33); G2: 11.1% (2/18) vs. 54.5% (18/33); G3: 5.6% (1/18) vs. 6.1% (2/33), Z=-2.766, P=0.006], were less likely to have serum
gastrin which was 10 times higher than normal [11.8% (2/17) vs. 56.0% (14/33), χ(2)=8.396, P=0.004], had higher probability of complication with type 2 gastric
neuroendocrine tumors (g-NET) [31.6% (6/19) vs. 3.0%(1/33), χ(2)=6.163, P=0.013], and had lower rate of liver
metastasis [21.1% (4/19) vs. 51.5% (17/33), χ(2)=4.648, P=0.031). There was no obvious difference between sporadic
gastrinomas and MEN1-related
gastrinomas in endoscopic findings. Both types presented enlarged and swollen gastric mucosa under the stimulation of high gastric acid, and multiple
ulcers in the stomach and duodenum could be seen.
Gastrinoma with type 2 g-NET presented multiple polypoid raised lesions in the fundus and body of the stomach. (68)Ga-SSR-PET/CT scan had a 100% detection rate for both types while (18)F-FDG-PET/CT scan had a higher detection rate for sporadic
gastrinoma compared with MEN1-related
gastrinoma [57.9% (11/19) vs. 20.0% (3/15), χ(2)=4.970, P=0.026]. Among the patients with sporadic
gastrinoma, 19 received surgical treatment, 1 underwent endoscopic submucosal dissection, 8 underwent transcatheter arterial embolization (TAE), and 5 underwent surgery combined with TAE. Among patients with MEN1-related
gastrinoma, 13 received surgical treatment, and the other 6 received
conservative treatment. The median follow-up of all the patients was 21.5 (1-129) months, and the 5-year survival rate was 88.4%. The 5-year survival rate of patients with sporadic and MEN1-related
gastrinomas was 89.5% and 80.0% respectively (P=0.949). The 5-year survival rate of patients with and without liver
metastasis was 76.2% vs. 100%, respectively (P=0.061). Conclusions: Compared with sporadic
gastrinoma, MEN1-related
gastrinoma has longer diagnosis delay, smaller
tumor diameter, lower
tumor grading, lower risk of liver
metastasis, and is more likely to complicate with type 2 g-NET, while there is no difference in survival between the two
tumor types.