Atypical carcinoid tumor of the larynx. A critical review of the literature.

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 immunohistochemistry 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.