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Salivary gland tumors are rare, clinically diverse neoplasms that represent less than 1% of all malignancies. In locoregional recurrent or metastatic disease, systemic therapy is the standard approach. While numerous small phase II studies have evaluated the activity of cytotoxic agents, either alone or in combination, the response rates are generally modest with objective response rates ranging from 15%–50%. Duration of response is cited in the range of 6–9 months. Given this, further evaluation of novel therapies is mandatory in these diseases. With the emergence of molecular targeted therapy, these tumors become optimal candidates for trials of investigational drugs and established drugs for new indications. Of note, given the often indolent nature of disease, only patients with progressive disease should be enrolled and treated on these clinical trials. Study designs must incorporate stringent inclusion criteria to enable accurate reporting of disease response and stabilization. With dedication and co-operation, patients with these rare neoplasms can be accrued to clinical trials and the establishment of new treatment guidelines will be forthcoming.
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