The intraoral lesion appeared as a 2 cm sessile, bluish mass located in the left anterior maxillary process. Palpation of the lesion did not seem to cause the infant any discomfort. A slight spongy feeling was suggestive of an absence of the buccal cortex. No thrill or pulse could be elicited from the mass. Although the lesion had supposedly expanded rapidly, the overlying mucosa was intact. (Figure 1)
Under sedation, advanced imaging, using computed tomography (CT), was performed. The axial CT scan revealed a 2 cm radiolucent lesion with irregular borders. The space-occupying lesion was causing the displacement of teeth and interrupting their normal development. (Figure 2)
Histopathologic examination revealed a nonencapsulated tumor showing local infiltration into the adjacent bone. The lesion showed a mixture of large polygonal cells and small hyperchromatic round cells which were arranged in sheets or alveolar structures. (Figure 3) A number of the larger cells contained melanin pigment. (Figure 4)
The histopathologic appearance of the specimen led the clinician to order a urinalysis. These results were normal for a young child, except for significantly elevated levels of vanillylmandelic acid (VMA). The VMA value was 3 standard deviations from the mean for a child in this age group.