Desmoplastic medulloblastoma

Case contributed by Jini P Abraham


Persistent vomiting, inability to walk and unable to see since 2 months. Opthalmological examination revealed bilateral chronic papilledema.

Patient Data

Age: 2 years
Gender: Female

Well defined altered signal intensity lesion noted in right cerebellar hemisphere along the tentorium. This is seen to cross the midline. Anteriorly, this is seen to compress the vermis and fourth ventricle, causing upstream dilatation of the ventricular system. Posteriorly, this is seen to cause mass effect over the medulla with inferior displacement of cerebellar tonsil. Posterosuperiorly, this is seen to abut the right transverse sinus. Diffusion restriction noted. Post-contrast study shows homogenous enhancement of the lesion. 

Obstructive hydrocephalus with transependymal CSF spread noted around the lateral ventricle.

Ascending transtentorial herniation noted on right side. Effacement of adjacent basal cisterns noted. 

Prominent CSF signal noted around the optic nerves bilaterally. 

Possibilities of medulloblastoma vs pilocytic astrocytoma are considered.

Case Discussion

Patient underwent midline suboccipital craniotomy and excision of the tumor. The tumor tissue was sent for histopathological evaluation.

Microscopic features show densely cellular tumor tissue composed of closely packed hyperchromatic primitive cells forming nodules and sheets. The nodules show hypocellularity and cells are larger with finely fibrillary background. The internodular areas show compact arrangement of cells with increased mitosis. The nodules are reticulin free and the internodular areas are reticulin rich.

Features suggestive of desmoplastic medulloblastoma.

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