Ectopic Cushing's Syndrome Unveiling a Metastatic Parotid Carcinoma. (15th October 2019)

• Record Type: Journal Article
• Authors: Castro Oliveira, Sofia; Neves, João Sérgio; Souteiro, Pedro; Belo, Sandra; Oliveira, Ana Isabel; Moreira, Helena; Mergulhão Gomes, Paulo; Coelho, Lígia; Sarmento, Cristina; Fonseca, Elsa; Neves, Celestino; Freitas, Paula; Carvalho, Davide
• Other Names: Kane Michael P. Academic Editor.
• Is Part Of: Case reports in endocrinology. Volume 2019(2019)
• Publication Date: 2019-10-15
• Subjects: Endocrinology -- Periodicals; Endocrinology -- Case studies -- Periodicals; Endocrine glands -- Diseases -- Periodicals; Endocrinology; Endocrine System Diseases; Endocrine glands -- Diseases; Endocrinology; Electronic journals; Periodicals; Case studies; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.4
• DOI: 10.1155/2019/3196283
• Languages: English
• ISSNs: 2090-6501

Abstract

Abstract : Introduction . Adrenocorticotropic hormone (ACTH) ectopic production is a rare cause of Cushing syndrome (CS). The most commonly associated tumours are small-cell lung carcinoma along with bronchial and thymic carcinoids. To date, only 5 cases have been published in the literature featuring ectopic ACTH secretion from metastatic acinic cell carcinoma (ACC) of the parotid gland. We hereby describe a very uncommon case of ectopic CS (ECS) unveiling a metastatic parotid ACC. Case Presentation. A 46-year-old man with hypertension and dyslipidemia diagnosed 4-months before, as well as new-onset diabetes mellitus unveiled 1-month earlier, was referred to emergency department for hypokalemia. Hormonal study and dynamic biochemical tests performed indicated ECS. Imaging and cytological findings pointed toward a likely primary right parotid malignancy with liver metastases. Somatostatin receptor scintigraphy has shown an increased uptake in the parotid gland and mild expression in liver metastasis. The patient underwent right parotidectomy, and histopathologic examination confirmed ACC. Meanwhile, hypercortisolism was managed with metyrapone, ketoconazole, and lanreotide. Despite chemotherapy onset, a rapid disease progression and clinical course deterioration was observed. Conclusion. The present report highlights a rare ECS, exposing a metastatic parotid ACC, with an aggressive and challenging clinical course, representing the first case whose diagnosis of ECS came prior to ACC.