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Vol. 33. Issue 6.
Pages 389-393 (November - December 2022)
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Vol. 33. Issue 6.
Pages 389-393 (November - December 2022)
Case Report
Diffuse leptomeningeal glioneuronal tumor: A review of diagnosis and management with an illustrative case
Tumor glioneuronal leptomeníngeo difuso: diagnóstico y tratamiento con un caso ilustrativo
Rajab Al-Ghanema,
Corresponding author

Corresponding author.
, Rafael Luque Baronab, Alicia Godoy-Hurtadoa, José Manuel Galicia Bulnesa, Osamah El-Rubaidia
a Servicio de Neurocirugía, Hospital Universitario de Jaén, Jaén, Spain
b Servicio de Anatomía Patológica, Hospital Universitario de Jaén, Jaén, Spain
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Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are a rare indolent neoplasm described in the 2016 WHO classification of tumors of the central nervous system (CNS). We describe a case of an 11 year old boy who initially presented intermittent headache, low back pain and communicating hydrocephalus, misdiagnosed as having tuberculous meningitis. Further clinical deterioration with seizures was observed and follow-up MRI showed further aggravation of leptomeningeal enhancement in the basal cisterns. Biopsy of the brain and leptomeninges revealed a diffuse leptomeningeal glioneuronal tumor. DLGNT should be considered in the differential diagnosis of conditions presenting as communicating hydrocephalus with nodular lesions and leptomeningeal enhancement. A timely histologic diagnosis through a biopsy of the brain is necessary to confirm the diagnosis.

Diffuse leptomeningeal glioneuronal tumor
Communicating hydrocephalus
Leptomeningeal enhancement
Epileptic seizures

El tumor glioneuronal leptomeníngeo difuso es una entidad infrecuente, con un curso indolente; fue descrito en la clasificación de los tumores del sistema del sistema nervioso central de la OMS 2016. Presentamos el caso de un varón de 11 años que comienza con un cuadro clínico inespecífico de cefalea, dolor lumbosacro e hidrocefalia comunicante. En el curso clínico aparecen crisis epilépticas con lesiones nodulares en RM craneal; fue diagnosticado de meningitis tuberculosa y tratado con tuberclostáticos. Ante un deterioro clínico progresivo, a pesar del tratamiento, y empeoramiento de los hallazgos en RM craneoespinal, se le realiza biopsia cerebral y de leptomeninges que confirma el diagnóstico de tumor glioneuronal leptomeníngeo difuso. El tumor glioneuronal leptomeníngeo difuso debe incluirse en el diagnóstico diferencial de los cuadros que se presentan con hidrocefalia comunicante y lesiones leptomeníngeas. Se precisa un diagnóstico histológico precoz mediante biopsia para establecer un tratamiento adecuado.

Palabras clave:
Tumor glioneuronal leptomeníngeo difuso
Hidrocefalia comunicante
Realce leptomeníngeo
Crisis epilépticas


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