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14 May 2014 - Case of the Week #312

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Case of the Week #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.

Micro images:

What is your diagnosis?

Malignant astroblastoma





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). 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:
Ependymoma: more fibrillar, nuclei smaller and less pleomorphic, true rosettes, less sclerosis; GFAP stains around blood vessels only; EMA has a dot-like or ring-like pattern (AJNR Am J Neuroradiol 2002;23:243, Childs Nerv Syst 2005;21:211)
Pilocytic astrocytoma: biphasic piloid areas with Rosenthal fibers alternating with spongy microcystic areas with eosinophilic granular bodies
Pleomorphic xanthoastrocytoma: fascicular pattern, pleomorphic cells, lipidized cells, eosinophilic granular bodies, perivascular lymphocytes

Treatment consists of resection for well differentiated tumors, but should be more aggressive for malignant tumors, which often recur (NIH - Genetic and Rare Diseases Information Center (GARD).

Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
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