ABSTRACT
Objective: to perform a retrospective analysis of 3 cases of Neurocutaneous Melanosis treated in our Department and to correlate the findings with literature.Description: neurocutaneous melanosis (NCM) is a rare congenital disorder characterized by the presence of multiple melanocytic nevi (3 or more), or a giant one, associated with benign or malignant proliferation of melanocytes in the central nervous system (CNS), with leptomeningeal infiltration. Diagnosis is based on clinical findings, MRI and pathology of lesions. With very bad prognosis, the disease is usually present with hydrocephalus, obstructive or not, or is associated with other CNS malformations such as Dandy- Walker complex. We are presenting three cases treated between 2005 and 2011, whose clinical expresion, prognosis and follow up matches with literature. Intervention: MRI, with and without contrast, were performed in all patients, as also surgical biopsy of meningeal lesions; VP shunt were placed for treating their hydrocephalus. Conclusion: the MNC is a poor prognosis disease with high mortality. The diagnosis is based on three pillars: complete clinical examination, MRI and histopathology of lesions. Specialists interaction (neurosurgeon - dermatologist - oncologist) is essential for the correct advice and support to parents.
Subject(s)
Hydrocephalus , MelanosisABSTRACT
Objetivo. Estudiar los signos clínicos, epidemiología, tratamiento, seguimiento y pronóstico de los meningiomas intracraneales en la infancia, y compararlos con la misma patología en la población adulta. Material y método. En nuestro Hospital se trataron 25 pacientes menores de 16 años con meningiomas, durante los últimos 20 años (1989-2009). En el estudio se contemplaron la edad y distribución por sexo, signos clínicos, neuroimágenes, localización de las lesiones, asociación con NF (neurofibromatosis), seguimiento, resección quirúrgica, subtipos histológicos, morbimortalidad y recidiva. Resultados. La relación hombre-mujer fué 1,1:1 (13:12) con una edad media de 10 años (11meses 15,6 años). Las crisis convulsivas fueron el síntoma más común (36%), seguido por la hipertensión endicraneana (HTE) (28%). Localización de la lesión: 15 supratentoriales, 6 en fosa posterior, 2 extracraneales y 1 espinal. Siete pacientes tenían asociada NF 1 ó 2. Todos los pacientes fueron operados. La mortalidad perioperatoria fué de 1 paciente y la mortalidad global de 3 pacientes, 2 de las cuales por eventos no relacionados al tumor (hemorragia digestiva síndrome de Steven Johnson) y 1 por progresión tumoral. Se logró la resección total (100%) en 16 pacientes, subtotal (60-99%) en 7 y parcial en 2 pacientes. En 14 pacientes mejorarron los síntomaspreoperatorios, en 6 no se modificaron, 4 empeoraron y 1 falleció. La recidiva fué 1/16 en resecciones totales, 6/7 en resecciones subtotales y 2/2 en resecciones parciales. Once pacientes necesitaron más de una cirugía. El meningioma meningoteliomatoso fue el subtipo más frecuente. Conclusión. Los meningiomas en pediatría son infrecuentes. No hay predominio de sexo como en la población adulta. La neurofibromatosis y radioterapia previa son los factores de riesgo más importantes. La resescción total es el factor pronóstico mas importante.
Objective. To study clinical signs, epidemiology, follow up, treatment and prognosis of intracranial meningiomas in childhood and correlates them with the same pathology in the adultpopulation. Material and method. 25 patients under 16 years with meningiomas were treated in our Hospital in the last 20 years (1989-2009). The study included their age and sex distribution, clinical signs, neuroimages, localization of the lesion, NF association, follow up, surgical removal, pathological subtypes of meningiomas, morbimortality and recurrence. Results. The male-to-female ratio was 1,1:1 (13:12) and themean age was 10 years ranging from 11 months to 15.6 years. Seizure was the most common symptom (36%) followed by intracranial hypertension (28%). Lesion localization: 15 supratentorial, 6 in the posterior fossa, 2 extracraneal and 2 spinal. Seven patient had NF 1/2 association. All patients were treated with surgery. Perioperative mortality was of 1 patient and the global mortality was of 3 patients, two caused by non-tumorrelated events (gastrointestinal hemorrhage Steven Johnson syndrome) and in the other case by tumor progression. Totalremoval (100%) was achieved in 16 patients, subtotal removal (60 to 99%) in 7 and partial in 2 patients. Preoperative symptoms improved in 14 patients, 6 remained unchanged, 4 had further deficit, and 1 died. The recurrence was 1/16 in total removal, 6/7 in subtotal removal and 2/2 in partial removal. Eleven patients required more than one surgery. Meningotheliomatous meningioma was the most frequent subtype. Conclusion. Meningiomas in childhood are infrequent. There is no gender predominance as in adults. The Neurofibromatosis and previous radiation treatment are the most important risk factors. Total removal is the most important prognostic factor.