|
Medullary
ganglioglioma -
a case report with review
of literature. Dr. Purav Patel,
Dept. Neurosurgery,
Apollo Hospitals, Chennai, India.
Brain stem
ganglioglioma is an uncommon primary neoplasm of the Central nervous
system. There are only 34 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. |
|
|
 |
| CT-plain |
CT-
contrast | |
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.
Pathologically, the
diagnosis requires both astrocytic and neuronal cell populations to
be present. Histologic diagnosis may be complicated due to
difficulty in determining whether the ganglion cells are truly
neoplastic or simply ‘trapped’ neurons in an infiltrating glioma.
According to Russel and Rubinstein, to be considered neoplastic,
neurons must be either clearly heterotopic (located away from gray
matter) or atypical, showing disorientation, bizarre shapes or
sizes, or binucleation.
Radical surgical
excision gives excellent outcome.
Ganglioglioma of the
brain stem are rare. For a lesion, particularly in children, with a
low intensity on T1-WI and high intensity on T2-WI involving the
medullarly region, ganglioglioma should be kept in mind as
differential diagnosis.
| 
