Sarcomas and sarcomatoid neoplasms of the major salivary gland regions. A clinicopathologic and immunohistochemical study of 67 cases and review of the literature.

Sixty-seven cases of sarcomas and sarcomatoid neoplasms of the major salivary gland regions were studied in order to determine the clinical and histomorphologic features and biologic behavior. Fifty-seven of these proved to be sarcomas and the two most common types were malignant schwannoma (11) and fibrosarcoma (9). Nine sarcomas could not be subclassified morphologically. Ten cases, originally believed to be sarcomas, proved by means of immunohistochemistry to be either carcinomas (five cases) or melanomas (five cases). Fifty-nine of the 67 cases occurred in the parotid gland regions, and the remaining eight occurred in the submandibular regions. Twenty of the 67 cases were thought to arise from within the gland, nine from paraglandular tissues, and insufficient data was present to anatomically categorize the other 38 cases. The mean age of occurrence was 42 years for men and 38 years for women. A swelling was the presenting symptom in 64 cases, with a mean duration of 4.3 months. Pain, tenderness, or paralysis were noted in 17 cases, but the swelling was painless in seven cases. Follow-up data of 42 sarcoma patients revealed that 17 experienced recurrences, 16 developed metastases, and 15 died of disease. These rates were lowest among patients with tumors arising from within the gland (Group I) and highest among those patients with tumors of paraglandular origin (Group III). Mean survival time for those dying of disease was 2.4 years, and a 5-year survival time appeared to be a significant indicator of cure. The most successful therapy was either parotidectomy (superficial or total) or a combination of surgery and radiation. The morphologic and the immunohistochemical evidence suggest that the majority of the tumors represent true sarcomas that may arise from undifferentiated pluripotential cells, but that the remainder (15%) represent epithelial malignancies.