Letter to “Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update”

Gosal, Jaskaran Singh; Tiwari, Sarbesh; Garg, Mayank; Bhaskar, Suryanarayanan; Jha, Deepak Kumar

doi:10.1007/s00381-020-04610-w

Cited by 2 publications

(3 citation statements)

References 3 publications

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“…Over 50% of patients present with headache (20), hydrocephalus (21,25) and focal neurological deficits. Glioneuronal tumors are by far the most common histological type of brain tumors requiring surgery for epilepsy management (26) and are therefore part of the "low grade epilepsy associated neuroepithelial tumors" family (26)(27)(28)(29).…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Age at presentation for DIA/DIG is infant to 33 years while for other types such as PGNT, RGNT and GNTNI, it is 12-70 years (7,8,20). In addition to seizures, patients can present with hydrocephalus (21,25), increased intracranial pressure as well as focal neurological deficits depending on tumor location, rate of growth and age. DIA/DIG presents usually, although not exclusively, in infants with increasing head circumference and seizure (31).…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Management involves seizure and symptom control, including resection when possible (12,13). In sub-totally resected disease and recurrent or grade II/III tumors, adjuvant treatment in the form of radiation and or chemotherapy may be administered; however, data on improvements in outcome are lacking (14)(15)(16)(17)(18)(19)(20)(21). Due to the indolent behavior of these tumors, late effects and survival are increasingly being examined (22)(23)(24).…”

Section: Introductionmentioning

confidence: 99%

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Glioneuronal Tumors: Insights into a Rare Tumor Entity

Krauze

2021

Gliomas

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Glioneuronal tumors are a group of rare neoplasms made up of neural and glial components in heterogenous proportions, generally exhibiting WHO grade I clinical behavior. These tumors affect infants, children and young adults, but are also described in adults and the elderly. They are strongly associated with seizures. Tumor subtypes described under the umbrella of glioneuronal tumors are actively evolving but to date comprise central, extraventricular and lipo-neurocytoma, desmoplastic infantile astrocytoma and ganglioglioma, diffuse leptomeningeal glioneuronal tumor, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, rosette-forming glioneuronal tumor of the fourth ventricle, rosetted glioneuronal tumor with neuropil-like islands, gangliocytoma, ganglioglioma, anaplastic ganglioglioma and paraganglioma. They vary in radiographic appearance, with some exhibiting large heterogenous solid/cystic masses. With large scale genetic and molecular analyses ongoing, classification continues to evolve. Seizure management and surgical resection represent the cornerstones of management, with the use of systemic agents and radiation lacking conclusive results. Optimal management requires multidisciplinary discussion including

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