Acinic cell carcinoma emerging as a paraneoplastic Cushing's syndrome: A systematic review

Abstract

Background: The purpose of this study was to systematically review all cases of acinic cell carcinoma (AcCC) associated with paraneoplastic ectopic ACTH-dependent Cushing's syndrome (CS-EAS). Methods: An electronic search was performed October 2020, using PubMed/MEDLINE and Science Direct databases. Results: The search resulted in a total of 9 cases. AcCC was only found in the parotid, and primary treatment included surgical excision (n = 9), neck dissection (n = 6) and radiotherapy (n = 6). High-grade transformation was reported in 2 cases. CS-EAS was diagnosed 6 months after AcCC treatment in 4 patients. Clinical features observed included hypokalemia (n = 9), myopathy (n = 7), edema and hypertension (n = 6), as well as cushingoid fat distribuition (n = 5). In 6 cases, disease-associated death was reported. Conclusions: Clinicians should be aware of this aggressive presentation of AcCC and should consider this diagnosis in patients with Cushingoid signs and symptoms.

Keywords

• Acinic cell carcinoma
• Parotid
• Cushing syndrome
• Ectopic ACTH
• Paraneoplastic
• High-grade transformation