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Vol. 33. Issue 4.
Pages 190-194 (July - August 2022)
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Vol. 33. Issue 4.
Pages 190-194 (July - August 2022)
Case Report
A rare central nervous system tumor of childhood with spongiform appearance on brain magnetic resonance imaging; primary diffuse leptomeningeal oligodendrogliomatosis
Un raro tumor del sistema nervioso central de la infancia con apariencia espongiforme en la resonancia magnética cerebral; oligodendrogliomatosis leptomeníngea difusa primaria
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Hande Gazeteci Tekina, Pakize Karaoğlub,
Corresponding author
pakizekaraoglu@gmail.com

Corresponding author.
, Elif Bolatc
a Çiğli Regional Education Hospital, Pediatric Neurology Clinic, İzmir, Turkey
b Dr. Behçet Uz Children's Hospital, Pediatric Neurology Clinic, İzmir, Turkey
c Ege University, Faculty of Medicine, Department of Neurosurgery, İzmir, Turkey
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Abstract

Primary diffuse leptomeningeal oligodendrogliomatosis is a rare fatal tumor of childhood. Symptoms usually occur when the tumor causes hydrocephalus. Brain magnetic resonance imaging (MRI) may be nearly normal in the early stages of the disease, while hydrocephalus and multiple leptomeningeal cysts with spongiform appearance may appear later on. One may consider the diagnosis when radiologic findings become apparent with multiple leptomeningeal cysts. However, failure to recognize the imaging findings due to the rarity of the disease may delay the diagnosis.

Here, we report a 3.5-year-old girl who presented with ataxia and vomiting and had a diagnosis of primary diffuse leptomeningeal glioneuronal tumor with remarkable brain MRI findings as diffuse multiple tiny cystic lesions on the brain and spinal cord. She benefited from radiotherapy and temozolomide treatment with remission of brain MRI findings.

Increasing the number of reported cases will enable the elucidation of the disease's pathogenesis and the development of treatment protocols.

Keywords:
Central nervous system tumor
Children
Primary diffuse leptomeningeal tumor
Resumen

La oligodendrogliomatosis leptomeníngea difusa primaria es un tumor fatal infrecuente de la infancia. Los síntomas generalmente ocurren cuando este causa hidrocefalia. La resonancia magnética (RM) cerebral puede ser casi normal en las primeras etapas de la enfermedad, mientras que la hidrocefalia y múltiples quistes leptomeníngeos con apariencia espongiforme pueden aparecer más adelante. Se puede considerar el diagnóstico cuando los hallazgos radiológicos se hacen visibles con la aparición de varios quistes leptomeníngeos. Sin embargo, el hecho de no reconocer estas evidencias en las imágenes debido a la rareza de la enfermedad puede retrasar el diagnóstico.

Aquí, presentamos el caso de una niña de 3,5 años con ataxia y vómitos que tenía un diagnóstico de tumor glioneuronal leptomeníngeo difuso primario con muchas lesiones quísticas diminutas difusas en el cerebro y en la médula espinal observadas en la RM cerebral. La paciente se benefició del tratamiento con radioterapia y temozolomida con remisión de los hallazgos de la RM cerebral.

El aumento del número de casos notificados permitirá dilucidar la patogénesis de la enfermedad y desarrollar protocolos de tratamiento.

Palabras clave:
Tumor del sistema nervioso central
Niños
Oligodendrogliomatosis leptomeníngea difusa primaria

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