A 5-year-old boy presented with head trauma. A CT showed an expansile, calcified mass (Fig. 1) arising from the right occipitotemporal region with mass effect and sclerosis. An MRI revealed an expansile, extra-axial mass with mixed signal intensity on T1-W and T2-W, hypointense signal on T2* and intense peripheral contrast enhancement (Fig. 2). During surgery, the mass adhered tightly to the dura and appeared dark brown to black. The diagnosis of melanotic neuroectodermal tumor was made.
Melanotic neuroectodermal tumors are rare melanin-containing neural crest origin tumors that mostly occur in infants. They occur in the maxilla (65–70%), mandible (6–10%), calvarium-dura anterior fontanelle or parietooccipital (10%), brain (5%) and rarely elsewhere. Most run a benign clinical course after surgical resection. If incompletely resected, they tend to recur and invade locally in 10–15% of cases. The brain tumors are usually malignant and infiltrative [1].
Reference
Smith AB, Rushing EJ, Smirniotopoulos JG (2009) Pigmented lesions of the central nervous system: radiologic-pathologic correlation. Radiographics 29:1503–1524
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Saleem, S., Altinok, D. Melanotic neuroectodermal tumor of the calvarium. Pediatr Radiol 40 (Suppl 1), 159 (2010). https://doi.org/10.1007/s00247-010-1747-y
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DOI: https://doi.org/10.1007/s00247-010-1747-y