• Rare osteoclastic and pigmented neoplasm, primarily affects jaws of infants
• Rapidly growing neoplasm
• Presents in first 4 months after birth
• M : F = 6 : 7

Site:

• Maxilla (common)
• Mandible
• Rare: Skull, long bones, epididymis, mediastinum

Clinical: Expanding blue-black lesion – causes facial asymmetry – displacement of lip and alter base of nose + destruction of alveolar bone

Pathology:

• Arise from neural crest cells associated with tooth germ
• Malignancy rare
• Can recur

Radiology: Multiple areas of radiolucency – associated with displacing teeth

Histology: Neuroblasts + pigment containing cells forming ducts and tubules

Diagnosis:

• FNA
• VMA (Vanillylmandelic acid) – monitor recurrence

Differential diagnosis:

• Myoblastoma
• Teratoma
• Vascular tumor
• Rhabdomyosarcoma

Management: Surgical excision (15-20% recurrence)

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