Atypical and aggressive diffuse leptomeningeal glioneuronal tumor in a young adult: A case report and review of the literature.
Brain tumor
Central nervous system tumor
DLGNT
Diffuse leptomeningeal glioneuronal tumor
Spine tumor
Journal
Surgical neurology international
ISSN: 2229-5097
Titre abrégé: Surg Neurol Int
Pays: United States
ID NLM: 101535836
Informations de publication
Date de publication:
2022
2022
Historique:
received:
22
12
2021
accepted:
25
04
2022
entrez:
8
6
2022
pubmed:
9
6
2022
medline:
9
6
2022
Statut:
epublish
Résumé
DLGNT is a rare tumor, commonly diagnosed in pediatric age; in most cases, the pathology presents a slow and indolent evolution. We present a case report of a young adult affected by DLGNT characterized by aggressive and atypical behavior. A 21-year-old male presented with mild paraparesis and hypoesthesia with a D2 level. MRI scan of the brain and spine showed a dorsal intramedullary lesion; a diffuse craniospinal leptomeningeal thickening was also present. After a week, the neurological status deteriorated rapidly with paraparesis worsening and onset of acute hydrocephalus. The patient underwent external ventricular drain positioning; a C7-D4 laminectomy was subsequently performed with partial tumor resection. Histological examination revealed a DLGNT with aggressive aspects (Ki67 30%). Postoperatively, the patient showed an immediate mild worsening of the lower limbs deficit. After a few days, severe further neurological deterioration occurred with progressive motor deficit to the upper limbs and ultimately respiratory failure. Mechanical ventilation was necessary and the patient was transferred to the ICU; during the following weeks, he developed tetraplegia and underwent ventriculoperitoneal shunt positioning. By the time, the histological diagnosis was available, the clinical status would not allow radiotherapy or chemotherapy. The patient deceased approximately 90 days after hospitalization due to respiratory complications. DLGNT is a rare tumor; diagnosis requires a high index of suspicion and confirmation with biopsy. Although most cases have an indolent course, some patients may have aggressive forms. High proliferation index, hydrocephalus occurrence, and massive craniospinal leptomeningeal spread appear to be associated with worse prognosis.
Sections du résumé
Background
UNASSIGNED
DLGNT is a rare tumor, commonly diagnosed in pediatric age; in most cases, the pathology presents a slow and indolent evolution. We present a case report of a young adult affected by DLGNT characterized by aggressive and atypical behavior.
Case Description
UNASSIGNED
A 21-year-old male presented with mild paraparesis and hypoesthesia with a D2 level. MRI scan of the brain and spine showed a dorsal intramedullary lesion; a diffuse craniospinal leptomeningeal thickening was also present. After a week, the neurological status deteriorated rapidly with paraparesis worsening and onset of acute hydrocephalus. The patient underwent external ventricular drain positioning; a C7-D4 laminectomy was subsequently performed with partial tumor resection. Histological examination revealed a DLGNT with aggressive aspects (Ki67 30%). Postoperatively, the patient showed an immediate mild worsening of the lower limbs deficit. After a few days, severe further neurological deterioration occurred with progressive motor deficit to the upper limbs and ultimately respiratory failure. Mechanical ventilation was necessary and the patient was transferred to the ICU; during the following weeks, he developed tetraplegia and underwent ventriculoperitoneal shunt positioning. By the time, the histological diagnosis was available, the clinical status would not allow radiotherapy or chemotherapy. The patient deceased approximately 90 days after hospitalization due to respiratory complications.
Conclusion
UNASSIGNED
DLGNT is a rare tumor; diagnosis requires a high index of suspicion and confirmation with biopsy. Although most cases have an indolent course, some patients may have aggressive forms. High proliferation index, hydrocephalus occurrence, and massive craniospinal leptomeningeal spread appear to be associated with worse prognosis.
Identifiants
pubmed: 35673647
doi: 10.25259/SNI_1255_2021
pii: 10.25259/SNI_1255_2021
pmc: PMC9168386
doi:
Types de publication
Case Reports
Langues
eng
Pagination
214Informations de copyright
Copyright: © 2022 Surgical Neurology International.
Déclaration de conflit d'intérêts
There are no conflicts of interest.
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