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Clinical Problem Solving
July 2014

Mass Originating From the Maxillary Alveolar Crest in an Infant

Author Affiliations
  • 1University of Minnesota Medical School, Minneapolis
  • 2University of Minnesota, Minneapolis
JAMA Otolaryngol Head Neck Surg. 2014;140(7):667-668. doi:10.1001/jamaoto.2014.632

An infant presented with a large mass protruding from the right maxillary alveolar ridge. The mass was first noted at 2 months of age and was initially thought to be an odontogenic cyst. The mass expanded rapidly over the next several months and was associated with intermittent increased work of breathing, feeding difficulty, and a 4.5-kg weight loss. Intraoral examination revealed a large, firm, gray mass originating from the right maxillary alveolar ridge (Figure, A). Contrast computed tomographic imaging showed an expansile multilobulated heterogeneously enhancing mass measuring 4.1 × 4.8 × 4.4 cm originating from the right maxillary alveolar ridge with displacement of the floor of the maxillary sinus. Within the mass there was a central “sunburst” representing new bone formation. The patient was initially taken to the operating room for open biopsy. The specimen consisted of small blue cells with a neuroblastic appearance and cuboidal cells with variable melanin pigmentation forming glandlike epithelial structures within a background of dense fibrous stroma (Figure, B and C). Immunohistochemical analysis revealed that both the epithelial and neural components were positive for neuron-specific enolase. The epithelial cells stained positive for cytokeratin AE1/AE3 (Figure, D), tyrosinase, and S-100, while the neuroblastic cells stained positive for synaptophysin with 20% Ki-67 positivity. The patient’s urinary vanillylmandelic acid levels were within reference range.

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