Long term results in adenoidcystic carcinoma

Background: Due to the discreet initial symptoms and the locally aggressive infiltration with perineural spread the adenoidcystic carcinoma (ACC) presents a special diagnostical and therapeutical challenge. Patients: in a retrospective study the forms of 54 patients were analysed, whose average age was 55.5 (24 - 77) years. Results: The sex ratio showed a slight female preponderance with 57% to 43%. The major salivary glands were affected in 26 cases. The exact distribution of the ACC was: parotid gland (n = 18), submandibular gland (n = 8), oral cavity (n = 10), paranasal sinuses (n = 11), nasopharynx (n = 4) and larynx (n = 3). The most common symptoms were a tumor-related swelling and pain which persisted for a duration of several months after final diagnosis could be established. Facial palsy was observed in 4 patients. Histological examination revealed a tubular subtype in 4 cases, in 28 cases a cribriforme and in 12 cases a solid subtype. No definitive differentiation was possible in 10 specimens. The skull base was infiltrated in 16 patients. Except one patient all 54 underwent surgical therapy. Postoperative radiotherapy was additionally given in 25 cases which was combined with a chemotherapy in 6 patients. Nevertheless ACC recurred in 60% of our patients. Lymphnode metastases were observed in 13 patients after a latency of 3.3 years in average, but predominantly pulmonary metastases as distant spread developed in 18 patients after 5.8 years significantly later. The overall survival ratewas 84.38% after 2 years, 75.90% after 5 years, 50.49% after 10 years and 20.11% after 20 years. Male sex, infiltration of the skull base and histological evidence of perineural and perivascular spread proved to be statistically significant factors for an unfavourable prognosis. Conclusions: Due to the uncommon biological behaviour with a slow growth on the one hand side and an aggressive local invasion on the other hand side the ACC can be regarded as a challenging malignant disease for the clinician whose adequate therapy does not allow any standardized regime. The tendency for recurrence even after a period clinically free of symptoms makes a life long follow-up mandatory.