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Glioblastoma - IDH-1 wildtype

Case contributed by David Mitchell
Diagnosis certain

Presentation

Dysarthria and left sided weakness

Patient Data

Age: 60
Gender: Male

3.4 cm right frontoparietal enhancing lesion with marked vasogenic edema and regional positive mass effect. 3.2 cm left frontoparietal partially calcified lesion with minor adjacent vasogenic edema and regional sulcal effacement. An additional smaller enhancing focus at the left superior frontal gyrus which measures 1.6 cm. 4 mm of midline shift to the left.

mri

Mass in the right inferior frontal gyrus extending into the white matter of the right corona radiata. Thick peripheral nodular enhancement, and central non enhancement. Central reduction of ADC value. Extensive surrounding edema. Ill-defined infiltrating mass in the left centrum semiovale. Intrinsic right T1 signal. No convincing enhancement. Surrounding T2 and FLAIR hyperintense white matter abnormality extends into the left pre and post central gyri, both of which are expanded. Intralesional calcification. Multiple additional small foci of ring enhancement. 7 mm of midline shift to the left. No hydrocephalus.

pathology

CLINICAL NOTES: (R) frontal lesion

MACROSCOPIC DESCRIPTION: 1. "Brain tissue": A piece of firm tan tissue

FROZEN SECTION DIAGNOSIS: High grade glioma. A1.

MICROSCOPIC DESCRIPTION: 1-3. Sections show a moderately cellular astrocytic glioma with extensive areas of tumor necrosis. There are multiple foci of microvascular proliferation. Tumor cells demonstrate marked nuclear pleomorphism with hyperchromasia and prominent nucleoli. Frequent mitoses are observed.

IMMUNOHISTOCHEMISTRY: GFAP positive Nestin positive (high) Nogo A negative IDH-1 R132H negative (not mutated) ATRX positive (not mutated) P53 positive P16 negative Topoisomerase labeling index 15%. 

DIAGNOSIS: The features are of glioblastoma IDH wild type (WHO Grade IV)

Case Discussion

The right frontal mass was biopsied and turned out to be a GBM IDH1 wildtype. The contralateral non-enhancing calcifed left centrum seimovale lesion was unusual and the possibility of a transformed oligodendroglioma was raised however the immunohistochemistry did not suggest that. Given the multiplicity and presence of calcification, metastases were considered such as lung, mucinous GI, papillary thyroid, or osteosarcoma. In a female, breast and mucinous ovarian primaries should also be considered.

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