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

Abstract

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.