Diffuse leptomeningeal glioneuronal tumor

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare, primary CNS tumor, classified as distinct entity in 2016^[1] and described as diffuse oligodendroglial-like leptomeningeal tumor of children in the 2016 classification of CNS neoplasms by the WHO.,^[2] Typically, it's considered juvenile tumors^[3] but can occur in adults,^[4] the average age of diagnosis is five years.^[3] It's characterised by wide leptomeningeal spread^[5] with male predominance,^[6] like histopathology of neurocytoma,^[7] oligodendrocyte-like cytopathology,^[8] bland appearance, and severe clinical behaviour.^[9][10] Children's basal cisterns and inter-hemispheric fissures are typically involved in plaque like subarachnoid tumors. A common related intraparenchymal lesion is a spinal lesion.^[11] However, in certain situations, superficial parenchyma or Virchow-Robin gaps were affected.^[12][13]
Molecular and genetic investigations frequently show a combination of KIAA1549 and the serine/threonine protein kinase BRAF gene, and also deletions of the short arm of chromosome number 1 and/or the long arm of chromosome number 19.^[14]

Clinical features

Patients with DLGNT presented with a variety of clinical manifestations, depending on the involved area of the disease, ranging from, numbness and seizure to hydrocephalus symptoms Irritability, headaches and vomiting.^[15][16] The progression of the disease is slow, however there have been reports of anaplastic transition.^[17][18]

Diagnosis

MRI reveals broad leptomeningeal enhancing and thickness, which is frequently most visible throughout the spine, brainstem, and posterior fossa.^[19]