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Published on: February 22, 2015
[A case of primary cerebellar glioblastoma in childhood]
Hidenori Endo1, Toshihiro Kumabe, Hiroyuki Kon
1Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Aoba-ku, Sendai-city, Miyagi 980-8574, Japan.
Insights
Pediatric primary cerebellar glioblastomas are rare and aggressive brain tumors. This case highlights their poor prognosis and tendency for rapid cerebrospinal fluid dissemination in children.
Area of Science:
- Pediatric neuro-oncology
- Central nervous system tumors
- Glioblastoma research
Background:
- Primary cerebellar glioblastomas are exceptionally rare in pediatric populations, with limited reported cases.
- This study details a case of a 7-year-old girl diagnosed with this rare tumor.
Observation:
- The patient presented with headache, increased intracranial pressure, and left cerebellar signs.
- MRI revealed a left cerebellar hemisphere mass with obstructive hydrocephalus and a pons lesion.
- Histological examination confirmed World Health Organization grade IV astrocytoma with high MIB-1 labeling index.
Findings:
- Despite gross total resection, adjuvant radiotherapy, and chemotherapy (ACNU), the patient experienced local recurrence and cerebrospinal fluid dissemination.
- Subsequent multi-agent chemotherapy and whole-brain irradiation failed to halt disease progression.
- The patient succumbed to the disease 10 months after admission.
Implications:
- Primary cerebellar glioblastomas in children portend a very poor prognosis.
- Cerebrospinal fluid dissemination is a common and aggressive feature of this tumor type in pediatric patients.
- Further research into novel therapeutic strategies is crucial for improving outcomes.
Abstract:
Primary cerebellar glioblastomas are exceedingly rare in childhood, with only 19 cases having been reported. We treated a 7-year-old girl with primary cerebellar glioblastoma, who rapidly deteriorated due to cerebrospinal fluid dissemination. The 7-year-old girl was admitted to our hospital with a history of headache for one month. On admission, increased intracranial pressure and left cerebellar signs were observed. Magnetic resonance imaging (MRI) revealed a ring-enhanced mass in the left cerebellar hemisphere and a low intensity lesion in the pons. The tumor had compressed the fourth ventricle and caused obstructive hydrocephalus. Gross total resection of the left cerebellar tumor was performed. Histological examination revealed nuclear atypia, mitoses, and necrosis, which satisfied the World Health Organizations histological criteria for grade IV astrocytoma. The MIB-1 labeling index was more than 60%. She was treated with adjuvant therapies consisting of 60.2 Gy local irradiation to the posterior fossa, including the brain stem lesion, and chemotherapy using 1-(4-amino-2-methyl-5-pyrimidinyl)methyl-3-(2-chloroethyl)-3-nitrosourea (ACNU). However, the patient developed of anorexia and vomiting 4 months after surgery, and MRI disclosed local recurrence at the left middle cerebellar peduncle and diffuse dissemination along the lateral ventricle wall. The patient was treated with three-drug chemotherapy using ifosfamide, cisplatin, and etoposide and 39.2 Gy of whole-brain irradiation. However, her condition deteriorated gradually and she died 10 months after admission (6 months after the onset of tumor recurrence). Primary cerebellar glioblastomas in children carry a very poor prognosis and tend to cause cerebrospinal fluid dissemination.
