Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review

Delayed diagnosis of diffuse leptomeningeal glioneuronal tumour in a young child presenting with communicating hydrocephalus

“…8 Patients with DLGNTs tend to have a worse prognosis if they have an elevated Ki-67, are over 9 years old, have evidence of hydrocephalus, have an intraparenchymal tumour and anaplastic histology. 9 Therapies for DLGNT are non-standardised and have historically included chemotherapy and/or radiation therapy. [10][11][12][13] Carboplatin, vincristine and temozolomide have been among the most commonly used regimens with reported stable disease or minimal response in some patients.…”

“…DLGNT MC-1 tumours tend to be diagnosed at a younger age and are less aggressive than DLGNT methylation class 2 (MC-2) tumours 8. Patients with DLGNTs tend to have a worse prognosis if they have an elevated Ki-67, are over 9 years old, have evidence of hydrocephalus, have an intraparenchymal tumour and anaplastic histology 9…”

Diffuse Leptomeningeal Glioneuronal Tumour

“…According to some authors, males are more often affected [ 1 ]. Owing to its rarity, the aetiology, natural progression and exact prognosis remain unknown [ 2 ]. Furthermore, there currently exists no WHO grade for this tumour [ 1 , 2 ].…”

“…Owing to its rarity, the aetiology, natural progression and exact prognosis remain unknown [ 2 ]. Furthermore, there currently exists no WHO grade for this tumour [ 1 , 2 ]. The tumour was only inducted into the WHO classification in 2016 [ 1 , 2 ].…”

“…Furthermore, there currently exists no WHO grade for this tumour [ 1 , 2 ]. The tumour was only inducted into the WHO classification in 2016 [ 1 , 2 ]. We present the case of a particularly advanced case of DL-GNT with prominent radiological features in a 14-year-old boy who had been diagnosed 10 years prior.…”