Extrapulmonary small cell carcinomas are rare tumors of the APUD system. They are most commonly seen in the esophagus with rare cases reported in the head and neck. We present the sixth reported case of primary small cell carcinoma of the tonsil. These tumors are usually widely disseminated initially. Aggressive locoregional treatment with radiation is the treatment of choice. Systemic treatment is needed for presumed metastatic disease. Surgery may be an adjunct treatment used for locoregional control. We thank Lillian Lawson for her technical support in the preparation of this manuscript.
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