Ectopic Cushing's Syndrome Unveiling a Metastatic Parotid Carcinoma

• Type: Article in International Scientific Journal
• Date: 2019

Authors

Oliveira, SC (Author) Other; Neves, JS (Author) FMUP; Souteiro, P (Author) Other; Belo, S (Author) Other; Oliveira, AI (Author) Other; Moreira, H (Author) Other; Gomes, PM (Author) FMUP; Coelho, L (Author) Other; Sarmento, C (Author) Other; Fonseca E (Author) FMUP; Neves, C (Author) Other; Freitas, P (Author) FMUP; Carvalho, Davide (Author) FMUP

Journal

Title: CASE REPORTS IN ENDOCRINOLOGY

Vol. 2019

Pages: 1-7

ISSN: 2090-6501

Indexing

ISI Web of Knowledge - 3 Citations

Scopus - 4 Citations

Other information

Authenticus ID: P-00R-88S

DOI: 10.1155/2019/3196283

Abstract (EN): 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.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 7