Presentation
Worsening headache
Patient Data
There is a mass lesion extending from the left parietal lobe to the temporal lobe containing solid and cystic areas with substantial vasogenic edema surrounding it. There is calcification in the solid part of the mass.
The lateral ventricle is compressed and there is a 1 cm shift to the right.
A mass lesion in the parietal-temporal lobe of the left cerebral hemisphere has cystic and solid areas. The solid component exhibits a magnetic susceptibility artifact consistent with coarse calcification, and has low ADC values suggesting high cellularity diffusion restriction. There is heterogeneous enhancement in the solid component and peripheral contrast enhancement in the cystic component.
MRS over the solid component is dominated by a large choline peak and low NAA, supporting the tumoral spectrum.
The patient went on to have a resection.
In the surgical material sent from the left parietal mass, the cells of the tumor show papillary, pseudopapillary or ribbon-like arrangement characterized by perivascular radially arranged neoplastic cells exhibited ATRX (+, no loss), Olig2 focal (+), EMA and D240 patchy(+) immunophenotype, while other markers were negative.
Ki 67 proliferation index was around %10 and low mitotic activity (<1/10HPF) was observed with PHH3.
The findings favor Astroblastoma; for a definite diagnosis, alterations in MN1 gene should be demonstrated by molecular tests.
Case Discussion
Astroblastomas are very rare tumors in childhood and early adulthood. These tumors have distinct radiological and histopathological features.
In this case, we observe several features of astroblastomas, including calcification and contrast of both components.
Histopathologic findings are consistent with astroblastoma, and for a definitive diagnosis, alterations in the MN1 gene should be demonstrated by molecular tests. Unfortunately, this is not currently available.
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