Pulmonary Carcinoid Tumors With Cushingʹs Syndrome: An Aggressive Variant or Not?

Background Adrenocorticotropic hormone (ACTH)-secreting pulmonary carcinoid is considered an aggressive variant of carcinoid tumors. Current knowledge is based upon a limited number of reports with few patients. Methods All patients with Cushingʹs syndrome (CS) resulting from pulmonary carcinoid (PC) who underwent pulmonary resection at our institution from November 1966 through April 1998 were reviewed. Results The group studied consisted of 10 males and 13 females. The median age was 39 years (range: 14–71). Pulmonary symptoms were present in 4 patients. Chest radiographs identified an abnormality in 13 patients (57%) and chest computerized tomography (CT) identified an abnormality in all 20 patients examined. Before pulmonary resection, hypophysectomy and bilateral adrenalectomy were performed in 7 patients (30%) each. Median time interval from presentation to pulmonary resection was 17 months (range: 1–228). Lobectomy was performed in 16 patients, segmentectomy was performed in 4 patients, and bilobectomy, pneumonectomy, and wedge excision was performed in 1 patient each. There were no operative deaths. Typical carcinoid was identified in 21 patients (91%) and atypical carcinoid was identified in 2 patients (9%). The median tumor diameter was 1.3 cm (range: 0.3–10). Nineteen patients (83%) underwent mediastinal lymphadenectomy and lymph node metastasis was found in 6 patients (32%) (N1 in 4 patients, N2 in 2 patients). The median follow-up was 78 months (range: 1–432). CS resolved in all of the patients. CS with PC recurred in 4 patients and CS alone recurred in 1 patient. Two patients underwent curative re-resection. Two patients, one with disseminated PC, died at last follow-up. Conclusions Despite long delays in surgical therapy, pulmonary resection for ACTH-secreting PCs results in a favorable prognosis. Anatomic resection with complete mediastinal lymphadenectomy decreases local recurrence. Although rare these tumors do not seem to be as aggressive a variant of typical carcinoid tumors as previously reported.