Ectopic production of adrenocorticotropic hormone by carcinoid tumors is relatively uncommon. This report describes a woman who had Cushing syndrome from the ectopic secretion of adrenocorticotropic hormone by a carcinoid tumor. Before her hospitalization the patients conditions was misdiagnosed as disease of connective tissue and thus the patient was treated inadequately. The untreated hypercortisolism caused bilateral pneumonia and sepsis. There are three instructive elements of this case: (1) the recognition of Cushing syndrome, (2) the association of Cushing syndrome with thymic carcinoma (3) the need to treat the hypercortisolism and its complications as well as the tumor.
In April 2007, a 23-year-old woman presented to hospital with following symptoms: general weakness, progressive hyperpigmentation and facial rounding. Physical examination revealed moon face, violaceous striae, easy bruising, hirsutism, podedema, hypertension, tachycardia; the auscultation revealed diminished breath sounds. Laboratory results showed hypokalemia, hypoproteinemia, hyperglycemia, and anemia. A random serum ACTH and cortisol levels revealed significant elevation (ACTH=387 pg/ml (N<46), Cortisol=1592 nmol/l (N 119618)). The computed tomography (CT) of the chest showed thymic tumor and bilateral multisegmental pneumonia. She was treated with aminoglutethimide, insulin, antihypertensive, potassium, albumin, antimicrobial therapy, ketokonazole. In spite of the treatment the patient developed an abscess of the right upper lobe of the lung. In July, the patient underwent surgery (thymectomy with right upper lobectomy). Histological examination revealed the presence of small-cell carcinoma with invasion to surrounding fatty tissue. Immunohistochemical staining defined the tumor as an ACTH-secreting (Ki67-14%). After surgery and further treatment with Octreotide and chemotherapy the patients symptoms completely resolved and the ACTH and Cortisol levels were normalized. For the last 6 months the patient has not been given any therapy, her general condition remains satisfactory.
This case demonstrates the successful diagnosis and treatment of the ACTH-ectopic tumor as well as hypercortisolism and its complications.
25 - 29 Apr 2009
European Society of Endocrinology