Small cell carcinoma of the maxilla

Small cell carcinoma (SmCC) in the oral and maxillofacial region is extremely rare high-grade malignant tumor. We report a case of a SmCC occurring in the maxillary gingiva. A 39-year-old man exhibited upper left gingival swelling that rapidly increased in size. Histopathological examination revealed proliferation of round cells with scant cytoplasm. Immunohistochemically, the tumor cells were positive for cytokeratin AE1/AE3, chromogranin A, synaptophysin, neuron-specific enorase (NSE), CD56, Leu 7, neurofilament and bcl-2, but negative for CD20, CD79a, S-100 and cyclin D1. Surgical resection and radical neck dissection were performed, and adjuvant chemotherapy combining CDDP and irinotecan (CPT11) was administered. Ten months after the operation, buccal lymph node metastasis and distant metastasis to the liver were found. Although irradiation therapy was performed, the liver lesion was unaffected by irradiation. Therefore, a further chemotherapy including etoposide was performed. The mass of the liver remained resistant to additional chemotherapy, and he subsequently died because of cachexia in spite of the partial hepatectomy.