ACTH‐secreting Pancreatic Neuroendocrine Neoplasms: A Case‐Series

Introduction

Ectopic Cushing's syndrome occurs rarely in patients with pancreatic neuroendocrine neoplasms. Early recognition of symptoms is critical given the high morbidity and mortality associated with Cushing's syndrome.

Methods

A database of pancreatic NENs seen at the Moffitt Cancer Center between 1/2008 and 4/2022 was reviewed and cases of ectopic Cushing's syndrome were identified. Information was extracted on tumor characteristics, clinical signs and symptoms, therapies, and outcomes.

Results

13 cases were identified, ranging in age from 16 to 65 years at initial time of diagnosis (median 42). 12 of 13 patients had metastatic tumors at presentation. All were well‐differentiated at diagnosis although 2 were described as transformed to poorly differentiated on re‐biopsy. 4 patients also experienced Zollinger‐Ellison syndrome. 3 patients underwent bilateral adrenalectomy to manage uncontrolled Cushing's syndrome. Median overall survival of was 56 months from time of initial cancer diagnosis but only 18 months from diagnosis of Cushing's syndrome.

Conclusion

Our analysis shows that ectopic Cushing's syndrome is a highly morbid condition when occurring in pancreatic NENs and is associated with aggressive disease. Bilateral adrenalectomy can be considered for syndrome control. To our knowledge, this is the largest institutional case‐series of ACTH‐ secreting metastatic pancreatic NEN.

This article is protected by copyright. All rights reserved.