Ganglioglioma of the cervical cord

Case contributed by A.Prof Frank Gaillard

Presentation

Gradual upper and lower limb weakness and sensory changes.

Patient Data

Age: Adult
Gender: Male

The upper cervical cord is expanded (opposite C1 and C2) by an eccentric mass which is high on T2 and iso-intense to cord on T1 weighted images. The region demonstrates no convincing contrast enhancement and no evidence of haemorrhage. There is evidence of previous surgery in the area. 

Annotated image

The tumour (yellow dotted line) is located eccentrically within the spinal cord ( * ). Evidence of prior surgery is noted posteriorly (orange arrows). 

The patient had already had a laminectomy and small biopsy. 

Histology

Microscopic Description: 

Sections show a neuropil in which there are increased numbers of neurons. Some of these neurons are oriented in various directions. Many of the neurons have a rounded appearance and some of the neurons have an eccentric-placed nucleus. The cellularity of the non-neuronal background is minimally increased and occasional clusters of glial cells are seen. The Bielschowski stain shows filamentous staining of the cell bodies of neurons which are large and have irregular shapes. There are occcasional structures reminescent of Rosenthal fibres. In some regions there are increased numbers of blood vessels.

Immunohistochemistry shows that the neuronal cell bodies are positive for synaptophysin and have a dense population of terminal boutons on their surface. Many of the neurons which are irregular in shape are positive for phosphorylated neurofilament. There is punctate granular positivity for chromogranin in many of the neurons. The glial fibrillary acidic protein stain shows staining of astrocytes and their processes in the background of the neuronal cell population. Immunohistochemisty for MIB-1 shows only occasional positive nuclei.

FINAL DIAGNOSIS: The lesion is unusual. It is only modestly hypercellular and the hypercellularity is due mainly to the presence of neurons which are somewhat atypical. These neurons stain for phosphorylated neurofilament and synaptophysin and have large numbers of terminal boutons on their surface. There are glia present which are few in number but occasional form small clusters. This case was shown to [...], an international authority on CNS tumors and it was felt that this is a ganglioglioma. 

Case Discussion

This mass demonstrated only minimal increase in size over 3 years of followup, with minor clinical deterioration.

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Case information

rID: 4535
Case created: 9th Sep 2008
Last edited: 4th Jul 2016
Inclusion in quiz mode: Included

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