Bronchial carcinoid tumors causing Cushing's syndrome: more aggressive behavior and the need for early diagnosis

Abstract

Background: The aim of this study was to revisit the characteristics and outcomes of adrenocorticotropin-secreting bronchial carcinoid tumor (BCT) responsible for Cushing's syndrome (CS).

Methods: We conducted a single-institution retrospective review of 14 patients who underwent pulmonary resection for BCT that presented as CS from October 1993 to November 2011.

Results: The group consisted of 8 male patients (57%) and 6 female patients. The mean age was 40 years (range, 16-63 years). Three patients (21%) underwent unnecessary adrenalectomy or hypophysectomy, or both, before diagnosis of the main cause. The mean interval between clinical presentation and the chest operation was 33 months (range, 3-136 months). Operations included 12 lobectomies (86%), 1 segmentectomy, and 1 wedge excision. All patients underwent radical lymph node dissection. Histologic examination showed 11 typical carcinoids (79%) and 3 atypical carcinoids. Twelve patients were classified pT1 (86%) and 2 patients were classified pT3 because of the presence of 2 tumors in the same lobe. Lymph node metastases were found in 7 patients (50%) (3 pN1 and 4 pN2). The mean follow-up was 59 months (range, 3-174 months). No recurrence was observed.

Conclusions: Early detection of adrenocorticotropin-secreting BCTs is challenging. However, it avoids adrenalectomy and unnecessary hypophysectomy, limits the deleterious effects of chronic hypercortisolism, and reduces the risk of metastasis. The high prevalence of lymph node involvement confirms the aggressiveness of these tumors and justifies anatomic resection and radical lymph node dissection. Under these circumstances, the prognosis remains favorable, even in cases of N2 disease.