Korean J Radiol.  2006 Jun;7(2):145-148. 10.3348/kjr.2006.7.2.145.

Parenchymal Neurocutaneous Melanosis in Association with Intraventricular Dermoid and Dandy-Walker Variant: A Case Report

• Kim YJ
• Won YD
• Kim KT
• Chang ED
• Huh PW

• KMID: 1782188
• DOI: http://doi.org/10.3348/kjr.2006.7.2.145

Abstract

Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

Keyword

Melanoma; Brain, growth and development

MeSH Terms

Tomography, X-Ray Computed
Neurocutaneous Syndromes/*epidemiology/radiography
Melanosis/*epidemiology/radiography
Male
Magnetic Resonance Imaging
Humans
Dermoid Cyst/*epidemiology/radiography
Dandy-Walker Syndrome/*epidemiology
Comorbidity
Cerebral Ventricle Neoplasms/*epidemiology/radiography
Arachnoid Cysts/epidemiology/pathology
Adult

Figure

• 

  Fig. 1 Neurocutaneous melanosis in a 27-year-old man. A. Noncontrast CT scan demonstrates a hyperdense mass with an adjacent cyst in the left temporal lobe. The CT scan also shows an irregular fatty mass (-105 HU) with marginal calcifications within the temporal horn of the right lateral ventricle. B, C. The axial T1-weighted (B) and T2-weighted (C) MR images show a left temporal lobe mass that is hyperintense on T1-weighted images and it is hypointense on T2-weighted images. There is a peritumoral cyst posterior to the main mass. The MR images also showed a mass in the right lateral ventricle, which appears homogeneously hyperintense on the T1-weighted images and heterogeneously hyperintense on the T2-weighted images; this is consistent with a dermoid cyst. The cystic encephalomalacia in the right temporal lobe is probably related to an early childhood insult. D. The right parasagittal T1-weighted MR image confirms the location of the right side mass within the temporal horn of the lateral ventricle. E. The midline sagittal contrast-enhanced T1-weighted MR image reveals hypoplasia of the inferior vermis and dilatation of the inferior fourth ventricle that communicates to the enlarged posterior fossa. F. The axial contrast-enhanced T1-weighted MR image shows mild enhancements of the wall of the peritumoral cyst in the left temporal lobe. Also noted is mild diffuse enhancement of the leptomeninges (arrows).

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