Seventy-one cases of adenoid cystic salivary gland carcinoma were reviewed according to treatment modality and clinical course. Thirty-six patients (51%) were treated by combined surgery and radiation therapy. The tumors were classified by their histologic patterns into tubular, cribriform, and solid forms. Distant metastases, in 52%, were the most frequent and ominous sources of failure. In 35% of cases, distant metastases developed despite local control at the primary site. In this group, the disease had a more fulminant course with shorter survival. Histopathologically, the cribriform subtype was associated with multiple local recurrences, greater local aggressiveness, and a poorer salvage rate as compared with the tubular subtype. Late onset of local recurrences and distant metastases was especially associated with the cribriform subtype. Overall prognosis in terms of distant metastases and survival was worst for the solid subtype. Control of local disease is best achieved with combined surgery and radiation therapy. The high incidence of distant metastases may not be affected by this regimen. The ultimate outcome of therapy is poorly predicted. Survival appears to be based on the pattern in which distant metastases develop. Overly aggressive and mutilating surgical approaches for these tumors are not recommended in many instances. The need for the development of new, more effective forms of therapy is emphasized.

The records of 71 patients with adenoid cystic carcinoma of the salivary glands were reviewed to determine the dose response relationships for this aggressive tumor. Local control after treatment was determined for all patients and analyzed with respect to extent of surgery and dose of radiation. Of 70 patients who were available for evaluation of local control, 28 (40%) had a local recurrence and 42 (60%) did not. The highest control rates were found in patients who underwent both radiation therapy and surgery. Patients who received a dose equal to or greater than 6,000 rad (60 Gy) in addition to surgery had significantly higher local control rates than those who received less than 6,000 rad (60 Gy). Distant metastases developed in 50% of patients regardless of local control, with the following distribution: 39% lung, 19% bone, and 10% disseminated soft-tissue metastases. Lymph node involvement was rare. Implications for combined modality treatment are discussed.

Between 1960 and 1980, 71 cases of adenoid cystic carcinoma (ACC) were reviewed according to treatment modality and clinical course. Histologic review of pathologic slides was performed to classify the tumors into their predominant histologic pattern (tubular, cribriform, solid). The predominant histologic patterns of the tumors were equally divided between tubular and cribriform. Very few were classified as a solid pattern. In the patients receiving the same type of therapy (surgery and irradiation), the cribriform and tubular variants of ACC demonstrated no difference in the rate of distant metastases and overall survival. The cribriform variant demonstrated a significantly worse prognosis in terms of local recurrence rate. The patients who had a solid histologic pattern of ACC appeared to have an overall worse prognosis in terms of distant metastases and long-term survival. The long-term survival of patients with ACC may be related to the development of distant metastases despite local control.

The records of 76 patients with adenoid cystic carcinoma of major and minor salivary gland origin seen at the Washington University Medical Center from 1963-1980 were reviewed to determine factors involved in local control, development of distant metastasis, and survival. Twenty-four (32%) patients were initially treated with surgery alone, 36 (47%) with combined surgery and irradiation, and 16 (21%) with irradiation alone. Local control after treatment was determined for all patients and was analyzed with respect to extent of surgery and dose of irradiation. Of 75 patients evaluable for local control, 30 (40%) failed with a median time of less than 5 years. There was no significant difference for patients treated vigorously for local recurrences after surgery. Patients who had a complete surgical resection (i.e., negative margins) survived significantly longer than patients treated by incomplete resection or biopsy alone followed by irradiation. Distant metastases developed in 50% of patients regardless of local control. Median survival after development of distant metastases was 40 months. There was no difference between major and minor gland sources for development of metastases. Lymph node involvement was rare. Our data indicate that complete and radical surgery results in the best survival. Combined modality therapy with surgery and irradiation optimizes the control of local disease. However, since distant metastases develop in spite of local control, in most instances we cannot recommend mutilating surgery for this disease entity.

The speculation whether juvenile laryngeal papillomatosis may transform into or be associated with epidermoid carcinoma is explored. We document a case of invasive laryngeal carcinoma arising in preexisting juvenile laryngeal papillomatosis. After multiple childhood laryngoscopies and a tracheotomy, a 54-year-old, 30-pack per year smoker, who had never received radiation therapy, developed a florid exophytic transglottic squamous cell carcinoma. Histologically, the invasive epidermoid carcinoma was surrounded by a field of papillomata with varying degrees of atypical changes. After total laryngectomy, isolated papillomata were found in the lower trachea. There were no cervical lymph node metastases. No postoperative radiation therapy was given. Persistent squamous papillomata in the tracheostomy site, the lower trachea, and the posterior pharynx were treated with the CO2 laser. We emphasize the need to maintain a high index of suspicion for malignancy. In addition, we review the problem of benign papillomata in the aerodigestive tract following laryngectomy.