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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 649

Neurocutaneous melanocytosis presenting in a teenager: A case report and review of the literature

1 Department of Radiation Oncology, Nizams Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
2 Department of Pathology, Nizams Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India

Correspondence Address:
L Pavan Kumar
Department of Radiation Oncology, Nizams Institute of Medical Sciences, Hyderabad - 500 082, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.138201

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Neuro cutaneous melanocytosis (NCM) is a non-familial, congenital disorder characterized by multiple congenital nevi and brain or leptomeningeal abnormal melanin deposits. Here, we present an adult onset NCM. A 17-year-old boy presented with headache and double vision for 1 month. Magnetic resonance imaging of the brain showed hydrocephalus and abnormal meningeal hyper intensities in supra and infratentorial regions predominantly in the posterior fossa. Para medullary region showed an 11 × 10 mm nodular contrast enhancing nodule. Resection of an intramedullary central nervous system lesion revealed melanoma while skin biopsy was benign melanocytic nevus. As per Kadonaga and Frieden criteria, a diagnosis of NCM was made. Planned for craniospinal irradiation by three-dimensional conformal radiotherapy with a dose of 36 Gy, in 18 fractions (2 Gy/fraction and 5 days in a week) along with steroids however patient progressed and developed quadriplegia with intradural metastasis.

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