Primary leptomeningeal melanocytosis in a 10-year-old girl: a challenging diagnosis with a poor prognosis

Article Article scientifique Rapport de cas Primary leptomeningeal melanocytosis in a 10-year-old girl: a challenging diagnosis with a poor prognosis eng 2011 author Brunsvig Kirsten author Zenobi Marie 565433 author Rilliet Bénédict 50793 author El Hassani Yassine 652763 author De Haller Raoul Michel 518005 author Ansari Djaberi Marc Georges 61572 author Lobrinus Johannes Alexander 647649 author Hanquinet Sylviane 133458 author Fluss Joel Victor 28801 1444-1448 10.1177/0883073811409749 21670389 0883-0738 Primary leptomeningeal melanocytosis is rarely encountered in the pediatric population. Despite being considered a nonmalignant condition, it uniformly carries a poor prognosis given the ineluctable progression of meningeal infiltration. The case of a previously healthy 10-year-old girl who was first thought to suffer from recurrent hemiplegic migraine is reported. She later developed insidious subacute hydrocephalus. Meningeal biopsy performed during shunt insertion demonstrated an unexpectedly large number of melanocytes consistent with meningeal melanocytosis. Subsequently, the child developed recurrent shunt dysfunction and showed evidence of malignant transformation. The steps to reaching the proper diagnosis are discussed, and the current literature on this rare clinical entity as well as on related central nervous system melanocytic lesions that can occur in the pediatric population is reviewed. Central Nervous System Neoplasms/complications/pathology/therapy Child Female Humans Hydrocephalus/etiology Melanocytes/pathology Meninges/pathology Migraine Disorders/complications Nevus, Pigmented/complications/diagnosis/pathology Dewey 616.0757 Dewey 616.8 Dewey 618 58 Département de pédiatrie, gynécologie et obstétrique 223 61 Département des neurosciences cliniques 226 62 Département de radiologie et informatique médicale 227 false 504 Groupe Hanquinet Sylviane (radiologie neuro-diagnostique et neuro-interventionnelle en pédiatrie 842 541 Groupe Menache Starobinski Caroline 881 391 Métastases du foie 657 564 Oncologie et hématologie pédiatrique 907 Journal of child neurology 26 11 f32c08af-cc98-4c0d-8650-ec8201277dc4 Article (Published version) Restricted Lobrinus JA J Child Neurol 2011, 26, 1444.pdf 342882 application/pdf restrictedAccess Faculté de médecine / Section de médecine clinique / Département de pédiatrie, gynécologie et obstétrique Faculté de médecine / Section de médecine clinique / Département des neurosciences cliniques Faculté de médecine / Section de médecine clinique / Département de radiologie et informatique médicale

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Primary leptomeningeal melanocytosis in a 10-year-old girl: a challenging diagnosis with a poor prognosis	Journal of child neurology			2011	611	0

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Zenobi, Marie