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Ann Thorac Surg 1990;50:52-57
© 1990 The Society of Thoracic Surgeons

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Articles

Management of the ectopic ACTH syndrome due to thoracic carcinoids

^a Thoracic Oncology Section, Surgery Branch, National Cancer Institute Bethesda, Maryland USA
^b Diagnostic Radiology Department; Developmental Endocrinology Branch, National Institute of Child Health and Human Development Bethesda, Maryland USA
^c The Surgical Pathology Laboratory, Clinical Center, National Institutes of Health, Bethesda, Maryland USA

^_* Address reprint requests to Dr Pass, Thoracic. Oncology Section, Surgery Branch, National Cancer Institute, NIH, Building 10, Room 2B07, Bethesda, MD 20892.

The association of a bronchial or thymic carcinoid as a source for the ectopic production of adrenocorticotropic hormone (ACTH) has been reported since 1957, with approximately 72 cases in the literature. These patients are characterized by young age, long duration of Cushing's syndrome because of the inability to find the ectopic source, and a high incidence of hypophysectomy or adrenalectomy without curing the disease. A substantial number of patients, upon discovery of the thoracic ectopic source, are also found to have malignant carcinoid tumors with lymph node metastases. Fifteen patients have been explored for a presumed intrathoracic source of ACTH at our institution since 1983 and 14 carcinoids (13 bronchial, one thymic) have been resected. Seventy-one percent ([equation]) of the patients appear cured with normal plasma ACTH levels 5 to 57 months after resection, despite a 50% incidence of positive lymph node disease. Management of these patients demands an aggressive evaluation to prevent unnecessary adrenalectomy or hypophysectomy and to allow earlier resections before these potentially curable malignancies metastasize. When the tumor is discovered, thorough exploration and complete lymph node mapping with resection must be performed.

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