Tufted angioma of the maxilla: a rare case with unique clinical presentation.

Tufted angioma is an uncommon benign vascular tumor usually presenting in early childhood and affecting mainly the skin. It has been associated with Kasabach-Merritt syndrome, a severe coagulopathy of poor prognosis. There have been very few published cases of oral tufted angiomas, and maxillary bone involvement has not been hitherto reported. We present a case of a 10-year-old Caucasian boy with an erythematous left maxillary gingival mass accompanied by subjacent hard tissue swelling. Radiologic evaluation revealed an irregular mixed radiolucent and radiopaque area in the left maxilla, accompanied by alveolar ridge erosion, cortical plate expansion, and displacement and divergence of the premolars. The histopathologic examination showed scattered irregular tufted lobules of variably sized vascular spaces inside the subepithelial connective tissue and among the underlying bone trabeculae, exhibiting a "cannonball" appearance. Immunohistochemical evaluation found positivity for CD31, CD34, and smooth muscle actin, and a final diagnosis of tufted angioma with osseous involvement was rendered. A thorough review of the pertinent literature revealed only 9 previously published intraoral tufted angioma cases, the salient demographic and clinicopathologic features of which are summarized.