title: Neurocutaneous melanocytosis presenting in a teenager: A case report and review of the literature.
abstract: 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.
authors.forename: I
authors.initials: I
authors.lastname: Monica
authors.affiliation: Department of Radiation Oncology, Nizams Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
authors.forename: L Pavan
authors.initials: LP
authors.lastname: Kumar
authors.forename: Megha S
authors.initials: MS
authors.lastname: Uppin
authors.forename: Kotiyala V
authors.initials: KV
authors.lastname: Jagannath Rao Naidu
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology*; D061888:Craniospinal Irradiation; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008548:Melanosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000532:radiotherapy*; D020752:Neurocutaneous Syndromes / Q000000981:diagnostic imaging / Q000473:pathology* / Q000532:radiotherapy*; D011859:Radiography