Mass Originating From the Maxillary Alveolar Crest in an Infant

Abstract

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.

Use of Technological Aids in the Resection of a Rare Maxillofacial Tumor of Infancy

“…Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that usually presents within the first year of life as a pigmented, rapidly growing mass. First described in 1918 by Krompecher, the tumor is most commonly located in the maxilla (70%), followed by the remainder of the craniofacial skeleton (20%) and other areas (10%) (Nelson and Thompson 2006; Selim et al, 2008; Butt et al, 2009; Manojlović et al, 2012; Marston et al, 2014). Melanotic neuroectodermal tumor of infancy is most often benign, yet locally aggressive with invasion, destruction, and distortion of surrounding tissues.…”

“…Histopathology demonstrates cells of neural crest origin that stain positive for neuron-specific enolase and vimentin. Tubular/alveolar formations of melanocytes, neuroblastic cells, and fibrous stroma further characterize the lesion (Cutler, 1981; Kapadia et al, 1993; Mast et al, 1999; Nelson and Thompson 2006; Selim et al, 2008; Béogo et al, 2013; Marston et al, 2014) Thus, MNTI is distinguished from other tumors of neuroectodermal origin including neuroblastoma and small round blue cell tumors.…”