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Free Content Primary Melanocytic Tumors of the Central Nervous System in Children: Imaging Features with Pathologic Correlation

Primary melanocytic tumors of the central nervous system are rare neoplasms arising from proliferation of multipotent melanin-containing neural crest cells found in the leptomeninges. This review describes the imaging appearance of the entire spectrum of primary melanocytic tumors of the brain and spine in the pediatric population, in correlation with histopathology, clinical features, and prognosis, using sample cases from our institution. These tumors are subclassified by the World Health Organization on the basis of disease distribution and pathologic aggressiveness into the following 4 subtypes: meningeal melanocytoma, meningeal melanoma, meningeal melanocytosis, and meningeal melanomatosis. They can occur in any age group, though the diffuse form of the disease is more prevalent in children and has an association with giant cutaneous melanocytic nevi. Imaging is characterized by hyperattenuation on CT and intrinsic T1 hyperintensity on MR imaging from the presence of melanin and/or hemorrhage. The focal forms usually manifest as dural-based hemorrhagic masses, and the diffuse forms demonstrate dural thickening and enhancement, with or without hydrocephalus. There is a predilection for the medial temporal lobes, posterior fossa, and upper cervical spine. The prognosis is often poor, with a high incidence of malignant transformation of benign disease.

Learning Objective: To describe the imaging appearance of the spectrum of primary melanocytic tumors of the brain and spine in the pediatric population and to review the histopathology, clinical, and prognostic data available

Keywords: CMN = congenital melanocytic nevi; GFAP = glial fibrillary acidic protein; WHO = World Health Organization

Document Type: Research Article

Publication date: April 1, 2022

More about this publication?
  • Neurographics is the peer-reviewed, quarterly educational journal of the American Society of Neuroradiology. The journal includes review articles as well as high-yield case reports that have been solicited from society meetings including the annual ASNR meeting as well as the American Society of Spine Radiology, the American Society of Pediatric Neuroradiology, the American Society of Functional Neuroradiology, and the American Society of Head and Neck Radiology meetings. Unsolicited educational review articles and case reports are also accepted for review at the discretion of the Editor-in-Chief. Submissions focusing on a pictorial approach to educational objectives are highly encouraged. The journal is open access and available online. CME credit is offered for reading review articles and completing quiz-based self-assessment activities through the ASNR Education Connection portal.


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