Published online Aug 6, 2022. doi: 10.12998/wjcc.v10.i22.7989
Peer-review started: January 21, 2022
First decision: March 23, 2022
Revised: April 3, 2022
Accepted: June 13, 2022
Article in press: June 13, 2022
Published online: August 6, 2022
Processing time: 181 Days and 21.1 Hours
Ectopic Cushing syndrome (ECS) is a rare condition commonly associated with neuroendocrine tumors (NET), mainly bronchial carcinoids. The association of paraneoplastic syndrome with Merkle cell carcinoma (MCC) is limited to individual case reports.
In this article we report an unusual and striking presentation of ECS in a patient with known metastatic MCC. An elderly patient presented with new onset severe hypertension, hyperglycemia and hypokalemia, muscle wasting, and peripheral edema. A diagnosis of adrenocorticotropic hormone dependent, non-pituitary, Cushing syndrome was established. Medical therapy inhibiting adrenal function was promptly started but unfortunately the patient survived only a few days after diagnosis.
The occurrence of an aggressive form of ECS in patients with NET should be recognized as an ominous event. To our knowledge, the association of this complication in a patient with MCC had not been reported.
Core Tip: Merkel cell carcinoma (MCC) is an uncommon but highly aggressive skin cancer with neuroendocrine features. Its incidence and mortality are increasing. We describe an elderly patient with a 2-year history of metastatic MCC, with no apparent cutaneous lesion at diagnosis, who presented with uncontrolled hypertension, diabetes mellitus, and hypokalemia. A diagnosis of ectopic Cushing syndrome was established. The occurrence of ectopic Cushing syndrome in patients with neuroendocrine tumor is a major cause of poor prognosis. To our knowledge, this is the first reported case of ectopic Cushing syndrome linked to the rapid progression of a metastatic MCC.