Cushing's syndrome due to ectopic adrenocorticotropic hormone production secondary to hepatic carcinoid: diagnosis, treatment, and improved quality of life. 2008

Susan G Coe, and Winston W Tan, and Thomas P Fox
Department of Internal Medicine, Mayo Clinic, Jacksonville, FL 32224, USA.

BACKGROUND We describe a previously healthy 40-year-old woman with Cushing's syndrome caused by adrenocorticotropic hormone (ACTH) secretion from metastatic carcinoid. METHODS Over a 2-year period, the patient had multiple hospitalizations for uncontrolled hypertension, hyperglycemia, and hypokalemia. She had transient flushing, rashes, and a rapid weight gain. In addition, she developed anasarca and had a nontraumatic hip fracture 1 month before presentation. Subsequently, a hypertensive crisis resulted in admission to the intensive care unit and fine-needle aspiration of a liver lesion. CONCLUSIONS A diagnosis of metastatic carcinoid was established. She was transferred to our hospital for further evaluation and management. On arrival, she had the signs of Cushing's syndrome. Despite extensive evaluation, her primary carcinoid tumor was not localized. She was treated successfully with bilateral adrenalectomy and octreotide. CONCLUSIONS This case illustrates how early recognition of the signs and symptoms of excess ACTH is important for prompt and appropriate treatment.

UI MeSH Term Description Entries
D008113 Liver Neoplasms Tumors or cancer of the LIVER. Cancer of Liver,Hepatic Cancer,Liver Cancer,Cancer of the Liver,Cancer, Hepatocellular,Hepatic Neoplasms,Hepatocellular Cancer,Neoplasms, Hepatic,Neoplasms, Liver,Cancer, Hepatic,Cancer, Liver,Cancers, Hepatic,Cancers, Hepatocellular,Cancers, Liver,Hepatic Cancers,Hepatic Neoplasm,Hepatocellular Cancers,Liver Cancers,Liver Neoplasm,Neoplasm, Hepatic,Neoplasm, Liver
D008303 Malignant Carcinoid Syndrome A symptom complex associated with CARCINOID TUMOR and characterized by attacks of severe flushing of the skin, diarrheal watery stools, bronchoconstriction, sudden drops in blood pressure, edema, and ascites. The carcinoid tumors are usually located in the gastrointestinal tract and metastasize to the liver. Symptoms are caused by tumor secretion of serotonin, prostaglandins, and other biologically active substances. Cardiac manifestations constitute CARCINOID HEART DISEASE. (Dorland, 27th ed; Stedman, 25th ed) Carcinoid Syndrome, Malignant,Carcinoid Syndromes, Malignant,Malignant Carcinoid Syndromes,Syndrome, Malignant Carcinoid,Syndromes, Malignant Carcinoid
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D002276 Carcinoid Tumor A usually small, slow-growing neoplasm composed of islands of rounded, oxyphilic, or spindle-shaped cells of medium size, with moderately small vesicular nuclei, and covered by intact mucosa with a yellow cut surface. The tumor can occur anywhere in the gastrointestinal tract (and in the lungs and other sites); approximately 90% arise in the appendix. It is now established that these tumors are of neuroendocrine origin and derive from a primitive stem cell. (From Stedman, 25th ed & Holland et al., Cancer Medicine, 3d ed, p1182) Argentaffinoma,Carcinoid,Carcinoid, Goblet Cell,Argentaffinomas,Carcinoid Tumors,Carcinoids,Carcinoids, Goblet Cell,Goblet Cell Carcinoid,Goblet Cell Carcinoids,Tumor, Carcinoid,Tumors, Carcinoid
D003480 Cushing Syndrome A condition caused by prolonged exposure to excess levels of cortisol (HYDROCORTISONE) or other GLUCOCORTICOIDS from endogenous or exogenous sources. It is characterized by upper body OBESITY; OSTEOPOROSIS; HYPERTENSION; DIABETES MELLITUS; HIRSUTISM; AMENORRHEA; and excess body fluid. Endogenous Cushing syndrome or spontaneous hypercortisolism is divided into two groups, those due to an excess of ADRENOCORTICOTROPIN and those that are ACTH-independent. Cushing's Syndrome,Hypercortisolism,Syndrome, Cushing,Syndrome, Cushing's
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000182 ACTH Syndrome, Ectopic Symptom complex due to ACTH production by non-pituitary neoplasms. Ectopic ACTH Syndrome,ACTH Syndromes, Ectopic,Ectopic ACTH Syndromes,Syndrome, Ectopic ACTH,Syndromes, Ectopic ACTH
D000315 Adrenalectomy Excision of one or both adrenal glands. (From Dorland, 28th ed) Adrenalectomies
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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