Carcinoma adenoide quístico de vías aéreas respiratorias: evolución y tratamiento

Abstract

Until recently, adenoid cystic carcinoma (ACC) has been considered to be a borderline disease between benign and malignant because of its low level of malignancy, slow growth and scarce aggressivity. ACC is now a histologically well defined tumor and is currently classified as malignant; it has a prolonged natural history, is infiltrative, and tends to recur locally and give rise to local-regional metastases or, more rarely, remote metastases. Its incidence in the lower airways is low (0.1% of all broncho-pulmonary neoplasms). Eight patients (6 women and 2 men) have undergone surgery for ACC since 1969. The initial clinical picture included long-term evolution and symptoms were usually produced by large caliber obstruction of the airways, mainly affecting the trachea and large bronchi. Three cases involved the trachea resection with end-to-end anastomosis in two cases, right pneumonectomy in two others (one of which also involved tracheal carina resection), and two lobectomies. After thoracotomy, resection of the tumor was deemed impossible in two cases. Postoperative mortality was 12% (1 patient). Complementary radiotherapy was provided in the two non-resectable cases, or when there was invasion of resected edges or regional metastasis involving ganglia. Total survival was 71.4% after two years, 57.1% after five years and 38% after ten years, after excluding postoperative exitus. When the trachea was involved, survival was 33.3% after two and five years and nil after ten years, with the longest survival 108 months. When bronchi were involved, survival was 75% at five and ten years. We conclude that ACC is a rare, locally invasive tumor that tends to local recurrence, but that survival after surgery is prolonged with or without adjuvant radiotherapy.