• Uncommon, rapidly growing mesenchymal tumor of jaw or skull in infants, neural crest origin (Cancer 1966;19:196), with biphasic histology of melanin containing cells and neuroblast-like cells (eMedicine)

• Also called melanotic neuroectoderrmal tumor of infancy
• Historically called melanotic progonoma or retinal anlage tumor based on suspected cell of origin

• Usually benign but may be locally aggressive or undergo malignant change

• 2 month old boy with maxillary tumor (Can J Plast Surg 2008;16:41)
• 4 month old girl with midline skull lesion (Case of the Week #165)
• 6 month old girl with maxillary tumor (J Oral Maxillofac Pathol 2010;14:29)
• 11 month old girl with maxillary tumor (Contemp Clin Dent 2012;3:108)

• Excision with negative margins
• Recur in 37%, metastasis in 7%, no prognostic factors have been identified (Fetal Pediatr Pathol 2006;25:59)

Maxilla

Anterior region of maxilla

2 month old boy with maxillary tumor

Maxilla

• Biphasic with tubular or alveolar patterns
• Large melanin-containing cells with abundant cytoplasm and pale nuclei, surrounding nests of smaller neuroblast-like cells possessing scant or fibrillar cytoplasm
• Minimal mitotic activity, no atypia

Case of Week #165

Maxillary tumor #1

#2

Various stains of mandible lesion

Anterior region of maxilla

6 month old female infant with expansile growth of maxilla

• Larger cells: keratin and HMB45, Melan A, Fontana stain for melanion pigment, rarely vimentin, Ki-67
• Smaller cells: CD57/Leu7 and NSE

• Both cell types are usually S100- (Am J Surg Pathol 1993;17:566)

• Pigmented cells contain melanosomes, neuroblast-like cells contain neurosecretory granules and cytoplasmic processes

• Neuroblastoma/other small round blue cell tumors or melanoma: typically lack the clinical presentation of a jaw or skull tumor in infants and a biphasic pattern; have prominent necrosis, atypia and mitotic figures

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