Somatostatin analogs in patients with Zollinger Ellison syndrome (ZES): an observational study.

Sara Massironi,Maurizio Quatrini,Alessandra Elvevi,Pietro Invernizzi,Federica Cavalcoli

doi:10.1007/s12020-021-02915-7

Abstract

Zollinger Ellison syndrome (ZES) is a rare syndrome caused by gastrin hypersecretion from a gastrinoma. Gastrinoma treatment has two goals: the control of acid hypersecretion and the control of tumor growth. While therapy for the syndrome is univocally based on proton pump inhibitors, the one for disease control is still debated. We here aimed at evaluating the role of somatostatin analogs (SSAs) in the control of tumor progression in a series of ZES patients. A retrospective analysis of a prospectively collected database of ZES patients, followed and managed from 1990 to 2019, was performed. The patients' clinical, pathological, treatment, and follow-up data were analyzed. Data regarding SSAs therapy start, dosage, duration, and side effects were collected. 33 patients with ZES were diagnosed. Fourteen patients (42%) had a grade 1 (G1) neuroendocrine neoplasm (NEN), five had G2 (15%), none had G3. Fifteen patients (45%) had metastatic disease. Overall, 12 (36%) underwent SSAs therapy. The median treatment duration was 36 months. Eight patients (67%) had a sustained response to SSAs, four (33%) showed an early progression, with a significant difference in terms of PFS between the patients with early and late progression (84 vs 2 months, p = 0.004). No differences in terms of OS and PFS were observed between the treated and non-treated patients, despite the proportion of metastatic patients was greater in the SSAs-treated group (75% vs 29% in the non-treated group, p = 0.01). Present data support the use of SSAs in ZES, considering that gastrinoma is mainly a well-differentiated low-grade tumor (G1 or G2), with a high expression of somatostatin receptors.

Highlights

Gastrin secreting tumors, or gastrinomas, are rare functioning neuroendocrine neoplasms (NEN), usually arising from the duodenum or the pancreas
No differences in terms of overall survival (OS) and progression-free survival (PFS) were observed between the two groups, despite the proportion of metastatic patients was greater in the SSA-treated group (75% vs 29% in the non-treated group, p=0.01)
Present data support the use of SSAs in Zollinger Ellison syndrome (ZES), considering that gastrinoma is mainly a welldifferentiated low-grade tumor (G1 or G2), with a high expression of somatostatin receptors

Summary

Introduction

Gastrinomas, are rare functioning neuroendocrine neoplasms (NEN), usually arising from the duodenum or the pancreas. Gastrin hypersecretion from gastrinoma is responsible for Zollinger Ellison syndrome (ZES) characterized by hypersecretion of gastric acid and severe recurrent peptic disease [1]. Gastrinoma occurs in one to three cases per million people in the USA annually, making them the second most common functional, pancreatic neuroendocrine neoplasm [2]. There are two therapeutic goals in the management of patients with gastrinoma: the control of gastric acid hypersecretion and the treatment of the tumor itself. While the therapy for syndrome control is univocal and based on proton pump inhibitors (PPI), which have significantly decreased the morbidity and mortality resulting from severe ulcer disease [4], the management of the neoplastic disease is still debated

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