Somatostatin Treatment for Ectopic ACTH Syndrome due to Pancreatic Neuroendocrine Tumors: Review of the Literature.

Da Zhang,Xiao Zhai,Shun-Da Du,Hua-Dan Xue,Qing-Xing Liu,Yu Xiao,Zhao-Lin Lu,Ming-Ming Hu,Zhao-Hui Zhu,Xian-Lin Han,Hui-Juan Zhu,Lin Lu,Xiao-Ping Xing,Giuseppe Reimondo

doi:10.1155/2022/6283706

Abstract

Objectives To analyze and summarize the effect of SSA treatment on EAS due to p-NETs (EAS-p-NETs). Methods Thirteen patients with EAS-p-NETs treated with SSAs at our center or described in the literature were included in this study. Clinical characteristics, laboratory data, imaging studies, histopathologic results, the effect of SSA treatment, and the prognosis of these EAS-p-NET patients were evaluated. Results Four males and 9 females with an average age of 42.9 years were included in the study. The mean duration of follow-up was 38.8 ± 28.2 months. As one of the combined treatment measures, SSAs controlled the levels of ACTH and cortisol in 9 of the 13 patients (69.2%). Partial response was observed in 3 patients (23.1%), stable disease in 2 patients (15.4%), and progressive disease in 6 patients (46.2%). The median time to tumor progression was 24 months, and the median overall survival was 61 months. The side effects of SSA treatment included temporary mild abdominal pain, diarrhea, gallstones, and cholecystitis. Conclusions As a supplemental therapy, SSA treatment led to clinical and biochemical improvement with a good safety profile in patients exhibiting EAS-p-NET with metastasis. However, tumor progression was inhibited by SSA treatment in only a few patients. Combined with other treatments, SSAs may improve the prognosis of patients with EAS-p-NETs.

Highlights

Cushing’s syndrome (CS) is a rare endocrine disease. e prevalence of CS ranges from 0.7 to 2.4 per million people per year [1]
10–20% of CS cases are due to ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), which originates from an ACTH-producing or corticotropin-releasing hormone (CRH)-producing neuroendocrine tumor outside of the pituitary [2]
We present a case of ectopic CRH syndrome stemming from a p-NET with liver metastasis successfully treated with octreotide long-acting release (LAR) and analyze the clinical characteristics and prognosis of 12 patients with EAS due to p-NETs treated with Somatostatin analogs (SSAs) in the literature to analyze and summarize the effect of SSA treatment on EAS due to p-NETs

Summary

Introduction

Cushing’s syndrome (CS) is a rare endocrine disease. e prevalence of CS ranges from 0.7 to 2.4 per million people per year [1]. 10–20% of CS cases are due to ectopic ACTH syndrome (EAS), which originates from an ACTH-producing or corticotropin-releasing hormone (CRH)-producing neuroendocrine tumor outside of the pituitary [2]. P-NETs are a group of heterogeneous diseases with varying tumor biology and clinical presentations. E first goal is to control the excess production of ACTH or CRH and relevant clinical symptoms of hypercortisolism. Another goal is to control tumor progression. A meta-analysis systematically showed an antiproliferative effect of SSAs in advanced gastroenteropancreatic neuroendocrine tumors (GEP-NETs) [7]. In the management of GEP-NETs, stabilization of tumor growth, rather than tumor shrinkage, is the most frequent response to treatment. SSAs stabilize tumor growth in 36–70% of patients with metastatic GEP-NETs [8]

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