Salivary gland malignancies are rare tumors that comprise multiple histologic entities with diverse clinical behavior. Mucoepidermoid carcinoma is the most frequent primary salivary malignancy, followed by adenoid cystic and acinic cell carcinoma. Although most salivary malignancies are asymptomatic, presentation with a rapidly enlarging mass may be accompanied by pain, functional neurologic deficits, soft-tissue invasion, or nodal enlargement. Assessment of clinical behavior and physical exam greatly contributes to diagnostic workup. Preoperative imaging, to include ultrasound, computed tomography, or magnetic resonance imaging, may assist with surgical planning. Limitations of preoperative fine-needle aspiration cytology mean that, in some cases, definitive histologic diagnosis may not be established until therapeutic surgery is undertaken. Treatment strategies rely on oncologic resection of the primary site with negative margins as well as adjuvant radiotherapy in patients with high-risk features, such as high-grade histology, advanced T class, or perineural invasion. Regional lymphadenectomy is recommended for involved nodal basins. Patients with clinically node-negative disease at high risk for occult nodal metastases may be considered for elective lymphadenectomy or radiotherapy. Use of chemotherapy in the adjuvant setting, in combination with radiotherapy, remains controversial. The rate of objective response to palliative chemotherapy in recurrent or metastatic salivary gland malignancy remains low. In studies that include a significant proportion of adenoid cystic carcinomas, whether disease stability represents an indolent disease process or the true effect of a therapeutic drug may be difficult to discern. Recognition of genetic alterations and protein expression unique to salivary malignancies presents exciting new opportunities for molecularly targeted therapy, although the response to molecularly targeted therapy in studies has been modest thus far.
The Rising Incidence of Major Salivary Gland Cancer in the United States