Brainstem ganglioglioma

Posted by Dr.Purav Patel Dr.Purav Patel
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Brainstem ganglioglioma  ganglioglioma is an uncommon primary neoplasm of the Central nervous system. There are less than 40 cases reported in the literature and of them only three cases have been studied with CT and MRI findings.

We present a case of medullary ganglioglioma with histology and imaging features.
 
This 13-year-old boy presented with a history of change in voice since two years, nasal regurgitation since one month, and with on and off breathing difficulty & dysphagia since one week.

Clinical examination revealed high pitched nasal quality of voice (as a consequence of soft palate dysfunction) and horizontal jerky nystagmus more on looking towards left side with fast component same side. There was diminished left corneal reflex, absent bilateral gag reflex with tongue & uvula deviated towards left side. Deep tendon reflexes were brisk with downward planter responses.

CT scan brain showed an ill defined iso to hypodense mass in the dorsal medulla extending into left cerebellum, foramen magnum with central contrast enhancement and displacing fourth ventricle to right side.


MRI brain revealed a solid-cystic mass in the dorsal medulla extending into left cerebellum, into the foramen magnum, displacing the roof of fourth ventricle to right side.


T1 weighted images showed a spectrum of signal ranging from isointense to hypointense, relative to surrounding neural tissue and areas of contrast enhancement with gadolinium.
T2 weighted images showed high signal intensity.

Suboccipital craniectomy and partial excision of tumor was done.



Histopathology revealed spindle shaped glial cells (open arrows)with spindle to oval shaped nuclei in fascicles. Ganglion cells (black arrows) had large vesicular nuclei with prominent nucleoli and occasional bizarre and binucleate forms. Focal perivascular aggregates and diffuse infiltrate of lymphocytes
were present. These findings were consistent with ganglioglioma.
Radiotherapy was given to the tumor bed over six weeks.
Lower cranial nerves improved with no difficulty in breathing or swallowing. Gag reflex present and voice is slowly improving.
 
Discussion

Courville in 1930 described the term ganglioglioma for the tumor arising from undifferentiated cells that became neoplastic yet eventually differentiated along both glial and neuronal lines to achieve the mature form of the cells. Gangliogliomas are unusual CNS tumors of children and young adults.  They represent 0.4 to 1.7 % of all CNS tumors. Cerebral hemispheres (temporal lobe) are more commonly involved followed by region of third ventricle, cerebellum, brain stem and spinal cord.