MANAGEMENT OF PATIENTS WITH SMALL-CELL CARCINOMA AND THE SYNDROME OF ECTOPIC CORTICOTROPIN SECRETION

Background. Small cell carcinoma (SCC) associated with clinical eviden ce of tumor corticotropin (ACTH) production is common, and management of this syndrome is difficult. The purpose of this retrospective analy sis is to describe clinical features, prognosis, and treatment results in patients with SCC and the syndrome of ectopic ACTH secretion to pe rmit formulation of management guidelines for these patients. Methods. Using tumor registry data and chart review, the authors identified pa tients with SCC and ectopic ACTH secretion treated over 11 years at tw o large teaching hospitals. They recorded clinical and laboratory data regarding the patients' tumors and their endocrine syndrome along wit h results of treatment for the malignancy and the hypercortisolism. Re sults. Ten patients with SCC and ectopic ACTH secretion were identifie d. These patients were initially seen with adverse prognostic features , including elevations of serum lactate dehydrogenase and extensive st age disease. Cytotoxic chemotherapy and standard doses of antiadrenal medications rarely controlled the paraneoplastic syndrome. Bacterial o r opportunistic infections, although not neutropenic, developed in mos t patients. Median survival of patients diagnosed with the paraneoplas tic syndrome at the same time as the initial diagnosis of cancer was 4 months. However, three patients whose cortisol secretion was controll ed survived longer than 6 months. Conclusions. Patients with SCC and e ctopic ACTH syndrome have a poor prognosis. However, in the minority o f patients whose hypercortisolism can be controlled with cytotoxic che motherapy combined with treatment to inhibit cortisol biosynthesis, ef fective palliation can be achieved.