Ectomesenchymal Chondromyxoid Tumor of the Anterior Tongue: Nineteen Cases of a New Clinicopathologic Entity

We present 19 cases of a previously undescribed myxoid tumor of the anterior tongue. These lesions occurred in nine women and 10 men aged 9 to 78 years (median, 32 years). Most tumors were seen as slow growing, painless nodules in the anterior dorsal tongue. The duration of growth ranged from a few months of 10 years. All tumors were treated by surgical excision, and two recurred. Microscopically, they exhibited a lobular proliferation of ovoid and fusiform cells, which often had multilobated nuclei and occasional foci of atypia, in a chondromyxoid background. Some tumors entrapped muscle or nerve fibers and had a tendency for blunt infiltration of adjacent tissue. The cells were diffusely and intensely immunore active for glial fibrillary acidic protein (GFAP) and cytokeratin but were decorated less frequently with antibodies for smooth muscle actin and S-100 protein. Reactivity for epithelial membrane antigen and desmin was not found. We believe these tumors fail to meet established clinicopathologic criteria for any existing myxoid neoplasms of the tongue, including nerve sheath myxoma, myoepithelioma, benign mixed tumor, ossifying fibromyxoid tumor of soft parts, extraskeletal myxoid chondrosarcoma, and glial and chondroid choristomas or heterotopias. Although the histogenesis of this neoplasm is unclear, we suspect that a cell of undifferentiated ectomesenchyme is the progenitor and suggest the descriptive term ectomesenchymal chondromyxoid tumor (ECT) of the the anterior tongue be adopted.