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Ann Thorac Surg 2005;79:1132-1136
© 2005 The Society of Thoracic Surgeons

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Original articles: General thoracic

Pulmonary Carcinoid Tumors With Cushing's Syndrome: An Aggressive Variant or Not?

Division of General Thoracic Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota

Accepted for publication July 14, 2004.

^_* Address reprint requests to Dr Nichols, Division of General Thoracic Surgery, Mayo Clinic, Rochester, MN 55905 (E-mail: nichols.francis{at}mayo.edu).

Presented at the Thirty-ninth Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 31–Feb 2, 2003.

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 Cushing's 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.