Atypical Carcinoid Tumor of the Larynx

Abstract
To better characterize the clinical and pathological features of the laryngeal atypical carcinoid (LAC), 127 of the published cases were analyzed. The LAC had a predilection for males (3 m:1 f), with a peak incidence in the 6th and 7th decades of life. Seventy-eight percent of patients with a relevant clinical history were smokers. Most tumors presented in the supraglottic larynx (96%) and the mean size of the measured primary lesions was 1.6 cm. The tumors were frequently argyrophil (97% of those so stained), rarely argentaffin (2 cases), and on immunohisto-chemistry were often reactive when stained for keratins (96%), chromogranin A (94%), and calcitonin (80%). Surgical resection was the principal modality of treatment. Of the 127 cases, metastasis to neck nodes were found in 43%, to skin or subcutaneous sites in 22%, and to distant sites in 44%. Of the 119 patients with follow-up, 49% died with tumor. The cumulative proportion surviving was 48% at 5 years and 30% at 10 years, and there were significantly worse survival rates among patients with tumors larger than 1 cm, and for patients developing tumor involvement of skin and subcutaneous tissues. Adjuvant radiation did not affect survival rates.