title: Ectopic adrenocorticotropic hormone syndrome caused by a carcinoid tumor of the thymus. A case report.
abstract: Ectopic ACTH secretion accounts for less that 10% of all causes of endogenous Cushing's syndrome. Carcinoids are rare thymic tumors, and when associated with ACTH hypersecretion display local or distant aggressive behavior. A 32-year-old woman was admitted to the Endocrinology Unit for obesity, moon face, facial hirsutism, hyperpigmentation, and secondary amenorrhea. Laboratory test confirmed the hypercortisolism and excess ACTH, while dexamethasone suppressive test was negative. Thorax computed tomography (CT) showed an antero-superior mediastinal tumor invading the pericardium and left mediastinal pleura. A complete resection through median sternotomy of the tumor, pericardium and left mediastinal pleura was performed. After a one-year symptom-free period, hypercortisolism recurred, confirmed by laboratory findings. Although no signs of local recurrence were seen on thorax CT, left internal mammary lymph nodes involvement and vertebral body metastases at C7 and LI were found. Refractory electrolyte disturbances could not be corrected resulting in severe cardiac arrhythmia and death from cardiac arrest. The reported case draws attention on the aggressiveness of ACTH-secralso due to the refractory electrolyte disturbances with fatal outcome.
authors.affiliation: Gr. T. Popa University of Medicine and Pharmacy, School of Medicine, The IIIrd Surgical Unit, Iaşi, Romania.
authors.forename: Lidia
authors.initials: L
authors.lastname: Ionescu
authors.forename: R
authors.initials: R
authors.lastname: Dănilă
authors.forename: Christina
authors.initials: C
authors.lastname: Ungureanu
authors.forename: Carmen
authors.initials: C
authors.lastname: Vulpoi
authors.forename: Delia
authors.initials: D
authors.lastname: Ciobanu
authors.forename: Cipriana
authors.initials: C
authors.lastname: Stefănescu
authors.forename: V
authors.initials: V
authors.lastname: Fotea
authors.forename: D
authors.initials: D
authors.lastname: Negru
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis* / Q000209:etiology*; D000328:Adult; D015992:Body Mass Index; D002276:Carcinoid Tumor / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D003480:Cushing Syndrome / Q000175:diagnosis / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009765:Obesity / Q000150:complications; D012008:Recurrence; D012307:Risk Factors; D013953:Thymus Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000601:surgery; D014883:Water-Electrolyte Imbalance / Q000209:etiology