title: 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.
authors.affiliation: Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore, Karnataka 560066, India. sunilvf@gmail.com
authors.forename: Sunil V
authors.initials: SV
authors.lastname: Furtado
authors.forename: Nandita
authors.initials: N
authors.lastname: Ghosal
authors.forename: Alangar S
authors.initials: AS
authors.lastname: Hegde
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000175:diagnosis*; D002530:Cerebellopontine Angle / Q000473:pathology; D035301:Cranial Fossa, Middle / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D020360:Neoadjuvant Therapy; D017600:Neuroectodermal Tumor, Melanotic / Q000175:diagnosis*; D013346:Subarachnoid Space; D015173:Supratentorial Neoplasms / Q000175:diagnosis; D013702:Temporal Lobe / Q000473:pathology; D014057:Tomography, X-Ray Computed