Abstract

Acinic cell carcinoma (ACC) with high-grade transformation is an aggressive subtype of salivary gland neoplasm. Only a few cases of adrenocorticotropic hormone (ACTH) production in metastatic ACC have been documented. We present a patient case of ACTH-dependent Cushing syndrome (CS) that developed 18 months after surgical resection of ACC. The original tissue sample was found to stain positive for ACTH, and the development of CS coincided with aggressive progression of ACC. Our case highlights the potential significance of ACTH staining in ACC and the development of CS as a signal of dedifferentiation, disease progression, and a poorer prognosis.

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