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Images in Neurology |

Symptomatic Neurocutaneous Melanosis in a Child

Puneet Jain, MD; Lakshminarayanan Kannan, MD; Atin Kumar, MD; Elanthenral Sigamani, MD; Vaishali Suri, MD; Noufal Basheer, MCh; Ashish Suri, MCh; Sheffali Gulati, MD
JAMA Neurol. 2013;70(4):516. doi:10.1001/jamaneurol.2013.2230.
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A 3-year-old boy presented with focal seizures for 3 months. He had severe headache associated with nonprojectile vomiting. There was no history of altered sensorium or diminished vision. He was developmentally normal. Family history was unremarkable. The patient was irritable. There was a giant congenital nevus in “bathing-trunk” distribution with multiple satellite nevi (Figure 1A). There was atrophy of the left leg. There were no focal motor or sensory deficits. Muscle stretch reflexes were normal. The rest of the neurological and systemic examination was unremarkable.

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Figure 1. A child with neurocutaneous melanosis. A, Giant congenital nevus in “bathing-trunk” distribution with multiple satellite nevi. B and C, T1 (B)- and T2 (C)-weighted axial magnetic resonance imaging of the brain at the level of the pons shows a parenchymal lesion in the left medial temporal lobe (arrows) that is hyperintense on T1-weighted imaging and heterogenous with areas of hypointensity on T2-weighted imaging. The lesion in the pons is hyperintense on T1-weighted imaging (arrowhead in part B) and isointense on T2-weighted imaging.

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Figure 2. Histopathology of the surgical specimen. A, Hematoxylin-eosin stain (original magnification ×400) shows an infiltrative neoplasm with irregular nests of closely packed malignant cells, some of which contain intracytoplasmic melanin (large arrow) and mitotic figures (small arrows). B, HMB-45 staining shows strong immunoreactivity (original magnification ×400). C, S-100 staining shows positive nuclear staining of the tumor cells (original magnification ×200).

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