Ectopic ACTH Secretion Secondary to Metastatic Acinic Cell Carcinoma of the Parotid Gland: A Case Report and Review of Current Evidence for Systemic Therapy

• Main Authors: Louise Wade MBChB, Paul Kitching MB BCh, FRCPath, Emma De Winton MBBS, FRCR
• Format: Article
• Language: English
• Published: SAGE Publishing 2020-05-01
• Series: Journal of Investigative Medicine High Impact Case Reports
• Online Access: https://doi.org/10.1177/2324709620918080

Acinic cell carcinoma is a rare, typically indolent, neoplasm that arises in the salivary glands. Metastatic disease is uncommon, occurring in around 10% of cases. We report the case of a 46-year-old male in whom the first sign of disseminated disease was increased skin pigmentation due to paraneoplastic Cushing’s syndrome. He underwent 3 cycles of chemotherapy with carboplatin and paclitaxel with no symptomatic improvement and a mixed response on imaging. There is no evidence that systemic therapy prolongs survival in metastatic acinic cell carcinoma, and we lack a consensus as to which treatment options are most beneficial. A summary of published evidence regarding choice of palliative chemotherapy regimens and response is discussed in relation to the case.