Medulloblastoma

Case contributed by Dr Ernest Lekgabe

Presentation

Headache and confusion.

Patient Data

Age: 35
Gender: Male
CT

Hyperdense mass in the midline cerebellum with obstruction of the fourth ventricle and resultant hydrocephalus.

MRI

Posterior fossa mass with apparent attachment to the roof of the fourth ventricle (superior medullary velum), restricted diffusion, and intermediate heterogeneous contrast enhancement. Obstruction of the fourth ventricle and resultant hydrocephalus.

Case Discussion

Resection was performed.

 

Histopathology:

 

Paraffin sections show fragments of a densely hypercellular tumour. Tumour cells have markedly pleomorphic nuclei, some with delicate processes and are arranged predominantly in diffuse sheets in a fibrous stroma. Scattered Homer-Wright rosettes are noted. An occasional mitotic figure is identified. There is no microvascular proliferation and no necrosis is seen. Attachment of leptomeninges is noted. The tumour cells are diffusely synaptophysin and CD56 positive. Patchy staining for NeuN and GFAP. 

Topoisomerase index (Cellular proliferation index ) is about 25%.

 

DIAGNOSIS:

Medulloblastoma (WHO Grade IV)

 

DISCUSSION:

Medulloblastoma can be divided into histologic subtypes:

  • Classic (Most common, more than two thirds)
  • Nodular/desmoplastic 
  • Anaplastic/large cell (Least common, less than 10%).

There are also four established molecular subgroups (based on molecular markers of the tumour), which are becoming more important to clinical practise:  WNT (wingless), SHH (sonic hedgehog) and the non WNT/SHH groups (groups 3 and 4). WNT has the best prognosis while group 3 has the worst prognosis.

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Case information

rID: 54923
Case created: 7th Aug 2017
Last edited: 3rd Oct 2017
Inclusion in quiz mode: Included

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