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

Abstract PURPOSE: Zollinger Ellison syndrome (ZES) is a rare syndrome caused by gastrin hypersecretion from a gastrinoma. Gastrinoma treatment has two goals: the control of the gastrin-excess state 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 SSAs in the control of tumor progression in a series of ZES patients.METHODS: 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.RESULTS: 33 patients with ZES were diagnosed. Fourteen patients (42%) had a G1 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 SSA, four (33%) showed an early progression, with a significant difference in terms of PFS between the two groups (84 vs 2 months, p=0.004). No differences in terms of OS and 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). CONCLUSION: 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.

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A Prospective Observational Study to Evaluate the Effects of Long-Acting Somatostatin Analogs on 68Ga-DOTATATE Uptake in Patients with Neuroendocrine Tumors

Journal of Nuclear Medicine ◽

10.2967/jnumed.119.226332 ◽

2019 ◽

Vol 60 (12) ◽

pp. 1717-1723 ◽

Cited By ~ 2

Author(s):

Anni Gålne ◽

Helen Almquist ◽

Martin Almquist ◽

Cecilia Hindorf ◽

Tomas Ohlsson ◽

...

Keyword(s):

Observational Study ◽

Neuroendocrine Tumors ◽

Prospective Observational Study ◽

Somatostatin Analogs ◽

Long Acting

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Ulcerogenic Tumors of the Pancreas

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100100676 ◽

1968 ◽

Vol 26 ◽

pp. 186-187

Author(s):

J. C. Garancis ◽

J. F. Kuzma ◽

S. D. Wilson ◽

E. H. Ellison

Keyword(s):

Endoplasmic Reticulum ◽

Tumor Cells ◽

Islet Cell ◽

Disease Process ◽

Central Core ◽

Islet Cell Tumors ◽

Opaque Zone ◽

Granular Form ◽

Zollinger Ellison Syndrome

It has been proposed that a gastrin-like hormone elaborated by non-beta islet tumors of the pancreas may be responsible for a fulminating ulcer diathesis. Subsequently, a potent gastric secretagogue was isolated from ulcerogenic tumors of the pancreas. This disease process is known now as “Zollinger-Ellison syndrome”.In our studies of two cases of Zollinger-Ellison syndrome, pancreatic lesions were identified as alpha islet cell tumors (Fig. 1). Tumor cells were fairly uniform. The sizes of the alpha granules were not significantly different, but their number and distribution varied greatly from one cell to another. Each granule consisted of a round, highly dense central core, separated from the limiting membrane by an opaque zone. The granular form of the endoplasmic reticulum was particularly prominent. Numerous mitochondria, round or elongated, were dispersed throughout the cytoplasm. Individual or clusters of lysosomes were observed in the majority of cells.

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