BRAINSTEM GLIOMA IN CHILDREN

brainstem glioma patient
A case of Brainstem glioma 
History

A boy 10 years old came to us in OPD with c/o-

Gradual loss of weight-6 mths
Cough & cold off & on- 6 mths
Loss of appetite-3 mths
Vomiting-3 mths
Convulsions- 7 days



On General examination

 Pt conscious
 Grossly emaciated wt 13 kg
 HR-88/min
 RR-24/min
 BP-90/60 mm hg



ptosis in patient with brainstem gliomaCNS examination 

Lt eye medial squint, 
ptosis,
nystagmus horizontal
hypertonia all 4 limbs 
Neck rigidity present
DTR brisk
Plantar equivocal B/L

RS,CVS,P/A- NAD



CT of patient with brainstem glioma 1
Investigations

CBC- Normal except for lymphocytosis

Fundus- Disc margins blurred

Guarded LP done showed Lymphocytosis, with RBCs, protein sugar normal

S.Na-WNL




CT of patient with brainstem glioma 2


CT Scan

axial CT showing hyperdense 
SOL involving brainstem (supratentorial) encroaching bilateral thalamus with dilatation of frontal horn of both lateral ventricles without midline shift

MRI- could not be done in this patient due to financial problems.








Let us briefly review Brainstem Gliomas

These tumors occur in the region between the aqueduct of Sylvius and the fourth ventricle (brainstem)


These tumors have a predilection to originate from the left side. Most are located in the pons; Brainstem gliomas are highly aggressive brain tumors.


Histopathology
Histopathologically, brainstem gliomas can range from WHO Grade 1 to 4. Grade 1, juvenile pilocytic astrocytoma, Grade 2, diffuse astrocytoma, Grade 3, anaplastic astrocytoma, and grade 4, glioblastoma multiforme. The grading is based on the presence of nuclear atypia, vascular proliferation, mitoses, and necrosis. Typically, the necrosis is seen in Grade 4


Epidemiology
Brainstem gliomas have been reported to make up 9.4% of intracranial tumors in children. Brainstem gliomas account for approximately 10-20% of all childhood brain tumors. 

Sex and Age distribution
Some reports have suggested a slight male preponderance.
Bimodal age distribution, peak incidence in first decade, second peak in the fourth decade.
3/4 th younger than 20 years.
Identified in children as young as 1 year

Clinical features (Yellow marked features were present in this case)

Anatomic location determines the pathophysiological manifestation of the tumor.


Common presenting symptoms include failure to thrive, double vision, weakness, unsteady gait, difficulty in swallowing, dysarthria, headache, drowsiness, nausea, and vomiting. Rarely, behavioral changes or seizures. Older children may have deterioration of handwriting and speech.


Pontine lesions usually present with any or all of the above signs and symptoms, depending on location and extension. 

Midbrain and lower brainstem/upper spinal cord signs and symptoms may be seen with extension of the neoplasm to involve these structures.

Tectal lesions typically present with headache, nausea, and vomiting.

Hydrocephalus is a common presentation, especially for tumors in periaqueductal or fourth ventricle outflow locations.

Physical examination
Common clinical findings constituting a triad of cranial nerve deficits, long tract signs and ataxia of trunk and limbs. 
Papilledema may be seen.
6 th and 7th cranial nerves are involved commonly. Facial sensory loss and a primary position, upbeating nystagmus may be seen.
Involvement of cranial nerve III or IV suggests a mesencephalic component.
Tectal lesions may present with diplopia reflecting an internuclear ophthalmoplegia.


Differential diagnosis
Arteriovenous Malformations
Ependymoma
Medulloblastoma
Metastatic Disease to the Brain
Tolosa-Hunt Syndrome

Laboratory Studies
Blood chemistry are not helpful as a rule,
Cerebrospinal fluid (CSF) examination is often important for differential diagnosis. The protein content of CSF may be elevated. Because of the risk of increased intracranial pressure due to obstructive hydrocephalus, caution in clinical and imaging assessment prior to lumbar puncture is stressed.

CT scan  
CT imaging is an appropriate choice when MRI is not available. 
CT identifies calcifications, cystic changes, and displacement of the ventricular system.

MRI
Diagnostic test of choice. MRI can differentiate vascular malformations and other processes that can be misdiagnosed as a brainstem glioma on CT scan. 
The typical MRI appearance of a brainstem glioma is an expansile, infiltrative process with low-to-normal signal intensity on T1-weighted images and heterogeneous high-signal intensity on T2-weighted images, with or without contrast enhancement.
MR spectroscopy has been used to help distinguish between tumor and nontumor lesions in the brain. An elevated choline peak suggests neoplasm.

Treatment
Treatment of brainstem gliomas has been frustrating; at this point, new therapies have yielded little benefit over conventional treatment with radiotherapy alone.
Adjuvant chemotherapy is not used in children because efficacy has not been proven. Data have suggested that preradiation chemotherapy may improve survival in pediatric diffuse intrinsic brainstem gliomas.
Bevacizumab is a VEGF receptor inhibitor, approved as monotherapy for recurrent glioblastoma multiforme in May 2009.
Focal radiotherapy is the cornerstone of treatment of brainstem gliomas and can improve or stabilize the patient's condition. Response to radiotherapy, in addition to the dose of radiation, depends on several variables such as tumor location, histologic type, and response to early treatment.
Author: Dr Sushmita Ghosh
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