Gliosarcoma

Heterogeneous signal intensity and contrast-enhancing (ring enhancement) periventricular mass lesion is demonstrated in the right occipital lobe measuring approximately 3.5 x 4.2 x 5.4 cm and showing mild restriction areas. There is an extensive perilesional vasogenic edema. Further area of separate contrast enhancement noted in the splenium on the right and another heterogeneous enhancing lesion is demonstrated in the right hippocampus and parahippocampal gyrus.

MICROSCOPIC DESCRIPTION:

The sections show a densely hypercellular tumor. This is composed of a mixture of plump spindle and epithelioid cells arranged in intersecting fasciculi and diffuse sheets. Both cell types show moderate nuclear and cellular pleomorphism. Frequent mitotic figures are identified and there are large areas of confluent necrosis. At the interface between tumor and adjacent brain there is a prominent angiocentric pattern with tumor cells forming sleeves around blood vessels.

IMMUNOHISTOCHEMISTRY

• GFAP positive in epithelioid tumor cells; negative in spindle cells
• EMA patchy strong positive staining in both spindle and epithelioid cells with perinuclear dot pattern in many cells
• Nestin positive - high
• ATRX positive (not mutated)
• IDH-1 R 132H negative (not mutated)
• MGMT negative (likely methylated)
• Cytokeratins CK7, CK20, CK5&6, AE1/AE3 all negative
• CDX2 negative
• SMA positive in blood vessels; negative in tumor cells
• Desmin patchy moderate staining in spindle cells
• Topoisomerase labeling index: Approximately 35%. Immunostaining present in nuclei of both spindle and epithelioid cells.

DIAGNOSIS: "Brain tumor": Gliosarcoma (WHO Grade IV) 

COMMENT: The presence of perinuclear dot immunostaining for epithelial membrane antigen (EMA) suggests that this gliosarcoma may have arisen in an anaplastic ependymoma. Otherwise the histopathological and immunohistochemistry features are those of conventional gliosarcoma.