Background: Small cell carcinoma (SCC) associated with clinical evidence of tumor corticotropin (ACTH) production is common, and management of this syndrome is difficult. The purpose of this retrospective analysis is to describe clinical features, prognosis, and treatment results in patients with SCC and the syndrome of ectopic ACTH secretion to permit formulation of management guidelines for these patients.
Methods: Using tumor registry data and chart review, the authors identified patients with SCC and ectopic ACTH secretion treated over 11 years at two large teaching hospitals. They recorded clinical and laboratory data regarding the patients' tumors and their endocrine syndrome along with results of treatment for the malignancy and the hypercortisolism.
Results: Ten patients with SCC and ectopic ACTH secretion were identified. These patients were initially seen with adverse prognostic features, including elevations of serum lactate dehydrogenase and extensive stage disease. Cytotoxic chemotherapy and standard doses of anti-adrenal medications rarely controlled the paraneoplastic syndrome. Bacterial or opportunistic infections, although not neutropenic, developed in most patients. Median survival of patients diagnosed with the paraneoplastic syndrome at the same time as the initial diagnosis of cancer was 4 months. However, three patients whose cortisol secretion was controlled survived longer than 6 months.
Conclusions: Patients with SCC and ectopic ACTH syndrome have a poor prognosis. However, in the minority of patients whose hypercortisolism can be controlled with cytotoxic chemotherapy combined with treatment to inhibit cortisol biosynthesis, effective palliation can be achieved.