Calvarial malignant melanotic neuroectodermal tumour of infancy presenting with widespread intracranial metastasis

The piamater, branchial arch derivatives and melanocytes, derivatives of the neural crest, are associated with rare, sporadic, non-inherited embryonic neuroectodermal dysplasia. We report a case of a 13 year-old girl with a malignant melanotic neuroectodermal tumour of infancy, an uncommon malignant extra-axial pigmented tumour with an aggressive clinical course. The clinical presentation, radiology, surgical management, adjuvant therapy are discussed along with a brief review of literature. The patient had widespread intracranial metastasis at presentation and rapidly deteriorated while on adjuvant therapy. A hyperdense extra-axial tumour on plain computed tomogram in a child could suggest a melanotic neuroectodermal tumour. Its malignant variant is associated with a poor prognosis when associated with widespread intracranial metastasis. (C) 2011 European Association for Cranio-Maxillo-Facial Surgery.