Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 16, 2022; 10(17): 5723-5731
Published online Jun 16, 2022. doi: 10.12998/wjcc.v10.i17.5723
Adrenocorticotropic hormone-secreting pancreatic neuroendocrine carcinoma with multiple organ infections and widespread thrombosis: A case report
Akihiro Yoshihara, Kota Nishihama, Chisa Inoue, Yuko Okano, Kazuhito Eguchi, Soichiro Tanaka, Kanako Maki, Valeria Fridman D'Alessandro, Atsuro Takeshita, Taro Yasuma, Mei Uemura, Toshinari Suzuki, Esteban C Gabazza, Yutaka Yano
Akihiro Yoshihara, Department of Diabetes and Endocrinology, Japan Community Health care Organization Yokkaichi Hazu Medical Center, Yokkaichi 510-0016, Mie, Japan
Kota Nishihama, Chisa Inoue, Yuko Okano, Kazuhito Eguchi, Soichiro Tanaka, Kanako Maki, Mei Uemura, Toshinari Suzuki, Yutaka Yano, Department of Diabetes, Metabolism, and Endocrinology, Mie University, Tsu 514-8507, Mie, Japan
Valeria Fridman D'Alessandro, Department of Immunology, Mie University, Tsu 514-8507, Mie, Japan
Atsuro Takeshita, Department of Immunology, Faculty and Graduate School of Medicine, Mie University, Tsu 514-8507, Mie, Japan
Taro Yasuma, Department of Immunology and Department of Diabetes, Metabolism, and Endocrinology, Mie University, Tsu 514-8507, Mie, Japan
Esteban C Gabazza, Department of Immunology, Mie University School of Medicine, Mie University, Tsu 514-8507, Mie, Japan
Author contributions: Yoshihara A and Nishihama K were responsible for clinical treatment, follow-up of the patient, and manuscript preparation; Inoue C, Okano Y, Eguchi K, Tanaka S, and Maki K were responsible for clinical treatment, follow-up of the patient, and data interpretation; Fridman D'Alessandro V contributed to data interpretation; Takeshita A, Yasuma T, and Uemura M were responsible for radiological and pathological investigation; Yasuma T, Suzuki T, Gabazza EC, and Yano Y were responsible for data interpretation, intellectual contribution, and manuscript preparation.
Informed consent statement: Written informed consent was obtained from the patient to publish clinical details and images when she was alive.
Conflict-of-interest statement: Yutaka Yano reports receiving lecture fees from Novo Nordisk. Other authors declared no conflict of interest concerning this case report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Esteban C Gabazza, MD, PhD, Full Professor, Department of Immunology, Mie University School of Medicine, Mie University, Edobashi 2-174, Tsu 514-8507, Mie, Japan. gabazza@doc.medic.mie-u.ac.jp
Received: October 23, 2021
Peer-review started: October 23, 2021
First decision: December 17, 2021
Revised: December 20, 2022
Accepted: April 9, 2022
Article in press: April 9, 2022
Published online: June 16, 2022
Abstract
BACKGROUND

Ectopic adrenocorticotropic hormone (ACTH)-secreting neuroendocrine tumors are rare diseases. Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis. Infections and coagulopathies have been reported as the cause of death. However, detailed clinical descriptions of the morbid complications of ACTH-secreting neuroendocrine carcinomas have not been reported.

CASE SUMMARY

A 78-year-old Japanese woman consulted a medical center due to systemic edema and epigastric discomfort. Laboratory analysis revealed hypercortisolemia with increased ACTH secretion without diurnal variation in serum cortisol level. An enhanced computed tomography (CT) scan revealed a 3-cm tumor in the pancreatic head. The cytological material from endoscopic ultrasound-guided fine-needle aspiration was compatible with ACTH-secreting pancreatic neuroendocrine carcinoma. The Ki-67 index was 40%. She was transferred to Mie University Hospital for surgical treatment. The patient was diagnosed with urinary tract infection, cytomegalovirus hepatitis, esophageal candidiasis, pulmonary infiltrates suspicious for Pneumocystis carinii pneumonia, peripheral deep vein thrombosis, pulmonary embolism, and disseminated intravascular coagulation. The multiple organ infections and thromboses responded well to antimicrobial and anticoagulant therapy. Radioisotope studies disclosed a pancreatic tumor and a metastatic lesion in the liver, whereas somatostatin receptor scintigraphy showed negative findings, suggesting the primary and metastatic tumors were poorly differentiated. A CT scan before admission showed no metastatic liver lesion, suggesting that the pancreatic tumor was rapidly progressing. Instead of surgery, antitumor chemotherapy was indicated. The patient was transferred to another hospital to initiate chemotherapy. However, she died four months later due to the rapidly progressive tumor.

CONCLUSION

ACTH-secreting pancreatic neuroendocrine neoplasm is a rare disease with a very poor prognosis. The clinical course and acute complications of the tumor remain unreported. Here we report the clinical course of a rapidly progressive case of ACTH-secreting pancreatic neuroendocrine tumor that developed infectious complications due to many types of pathogens in multiple organs, widespread thromboses, pulmonary embolism, and disseminated intravascular coagulation.

Keywords: Neuroendocrine tumors, Cushing's syndrome, Ectopic adrenocorticotropic hormone syndrome, Pneumocystis pneumonia, Pulmonary embolism, Infections, Case report

Core Tip: Adrenocorticotropic hormone (ACTH)-secreting pancreatic neuroendocrine tumor is a rare malignant disease with a poor prognosis. The condition is frequently associated with infectious and thrombotic complications. However, the detailed clinical course and acute complications of the tumor remain unreported. Herein, we report a rare case of ACTH-secreting pancreatic neuroendocrine tumor associated with infections due to multiple pathogens in several organs and systemic coagulopathies. The infectious and thrombotic complications responded well to antibiotics, antiviral and antifungal drugs, and anticoagulants. However, radioisotope studies showed that the tumor was poorly differentiated, rapidly progressive with multiple metastatic lesions in the liver. On this basis, instead of surgical treatment, antitumor chemotherapy was indicated. Unfortunately, the patient died due to systemic tumor dissemination.