A patient with ectopic cortisol production derived from malignant testicular masses

• Published in: Nature clinical practice. Endocrinology & metabolism Vol. 4; no. 12; pp. 695 - 700
• Main Authors: Jain, Shilpa H, Sadow, Peter M, Nosé, Vania, Dluhy, Robert G
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.12.2008; Nature Publishing Group
• Subjects: Abdomen; Adrenal Rest Tumor - complications; Adrenal Rest Tumor - diagnosis; Adrenocortical Carcinoma - blood; Adrenocortical Carcinoma - diagnosis; Adrenocortical Carcinoma - pathology; Adrenocorticotropic Hormone - blood; Aged; Case studies; case-study; Complications and side effects; Cushing syndrome; Cushing Syndrome - blood; Cushing Syndrome - diagnosis; Cushing Syndrome - drug therapy; Cushing Syndrome - pathology; Diabetes; Diagnosis; Dosage and administration; Drug therapy; Edema; Endocrinology; Hormones; Hospitals; Humans; Hydrocortisone - blood; Hypertension; Hypokalemia; Hypoxia; Male; Medical education; Medical imaging; Medicine; Medicine & Public Health; Metabolism; Metyrapone - therapeutic use; Mitotane; Mitotane - therapeutic use; Oncology; Pathology; Potassium; Risk factors; Testicular cancer; Testicular Neoplasms - complications; Testicular Neoplasms - diagnosis; Ultrasonic imaging; United States
• ISSN: 1745-8366; 1759-5029; 1745-8374; 1759-5037
• DOI: 10.1038/ncpendmet0985

This article describes a patient with Cushing syndrome presumably attributable to an adrenocortical carcinoma arising from testicular adrenal rest cells. The authors discuss treatment options for adrenocorticotropic hormone-independent hypercortisolism and highlight the difficulties in determining the origin of ectopic cortisol production. Background A 65-year-old man presented to an oncology clinic with bilateral testicular masses, lower extremity edema, and cushingoid appearance. Investigations Measurements of serum cortisol and adrenocorticotropic hormone levels, testicular ultrasound and abdominal CT scans, and review of histopathology to identify the cellular origin of the ectopic cortisol production. Diagnosis Cushing syndrome was diagnosed on the basis of a markedly elevated 24-hour urine free cortisol level and classic cushingoid features. The etiology of Cushing syndrome was determined to be an adrenocortical carcinoma arising from testicular adrenal rest cells. Nevertheless, the possibility of a malignant Leydig cell tumor with ectopic cortisol production could not be excluded. Management Mitotane and metyrapone were used to decrease cortisol production. Excess mineralocorticoid activity was blocked with spironolactone; sodium retention was also managed with sodium restriction and diuretics. Despite initial success with this regimen, the patient died as a result of tumor progression and complications of poorly controlled hypercortisolism.