Published online Jun 16, 2022. doi: 10.12998/wjcc.v10.i17.5723
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
Processing time: 229 Days and 1.9 Hours
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.
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.
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.
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.