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14 May 2014 - Case #312

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Thanks to Dr. Joseph Fullmer, Upstate Medical University (USA) for contributing this case and part of the discussion.




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Case #312

Clinical history:
A 1 day old boy had a prior ultrasound at 36 weeks showing a 7 x 6 cm cerebral fluid collection, with echogenicity suggestive for brain hemorrhage. Postnatal MRI on Emerson showed a large left hemispheric heterogeneous mass, occupying the posterior two - thirds of the left hemisphere, causing significant mass effect with midline shift and brainstem compression. The infant was taken immediately for surgery and a biopsy was obtained.

Microscopic images:



What is your diagnosis?

Click here for diagnosis and discussion:


Diagnosis: Malignant astroblastoma

Immunostains:

EMA

GFAP

Trichrome



Discussion:
Astroblastoma is a rare (< 3% of primary brain gliomas), compact glial neoplasm with perivascular pseudorosettes formed of GFAP+ cells arranged around central, often sclerotic, blood vessels (Wikipedia: Astroblastoma [Accessed 22 March 2024]). No WHO grade is assigned to this entity. It typically affects children and young adults, with a median age of 11 years.

Grossly, it is often a well circumscribed, peripheral, cerebral hemispheric mass that is firm and often cystic. Microscopically, it is well circumscribed with discrete pushing borders. High grade lesions may be infiltrative. Most astroblastomas have perivascular pseudorosettes resembling ependymoma, but with thick processes from the cell body to the adventitia of the vessel. Vascular hyalinization is common, with little fibrillar background. It is recommended to limit this diagnosis to tumors with a predominance of these features, which may be present focally in other tumors. High grade / malignant astroblastomas, such as this tumor, have hypercellular and mitotically active regions, often with vascular proliferation or necrosis with pseudopalisading. EMA highlights the cell membranes, GFAP stains tumor cells and trichrome stains vascular sclerosis.

The differential diagnosis includes:
Treatment consists of resection for well differentiated tumors but should be more aggressive for malignant tumors, which often recur (NIH: Astroblastoma [Accessed 22 March 2024]).


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