Gliosarcoma IDH wild-type

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

New daily headaches, left side, for months.

Patient Data

Age: 55 years
Gender: Female
mri

A solid / cystic mass is present in the left posterior temporal / occipital lobe adjacent to the occipital horn of the lateral ventricle. I has a rounded solid enhancing component with ADC values similar to normal white matter. An adjacent cystic component is present with peripheral enhancement. Surrounding this, is a mantle of vasogenic edema.

pathology

Sections show fragments of a hypercellular astrocytic glioma. Tumor cells show marked nuclear and cellular pleomorphism with a biphasic spindled and epithelioid morphology. Frequent mitotic figures are identified. No heterologous elements are seen. There is prominent microvascular proliferation with multilayering of atypical cells around vessel lumena. Extensive areas of tumor necrosis are also present.

Immunohistochemistry results show tumor cells stain:

  • GFAP
    • Positive epithelioid cell component
    • Negative spindle cell component
  • Nestin Positive (high)
  • IDH-1 R132H Negative (not mutated)
  • ATRX Positive (not mutated)
  • MGMT Positive (non methylated)
  • p53 Positive
  • p16 CDKN2A Positive
  • Topoisomerase labeling index: Approximately 20%.

DIAGNOSIS: Gliosarcoma, IDH-1 wild type (WHO Grade IV).

Case Discussion

Gliosarcomas are unusual tumors, difficult if not impossible to distinguish from the far more common glioblastoma on imaging. 

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