Primary Cerebral Pleomorphic-Myxoid Sarcoma With Many Hyaline Globules

A case of primary sarcoma that arose in the right temporal lobe of a 59-year-old man is reported. The patient had a history of resection of a "pituitary tumor" and postoperative radiotherapy about 40 years earlier. The cerebral tumor consisted of loose and diffuse proliferation of large pleomorphic cells in a matrix showing a markedly myxoid change and containing a variable amount of delicate collagen fibers. Some tumor cells had a large intracytoplasmic vacuole containing acid mucopolysaccharide, thus featuring "pseudolipoblasts." Differentiation along other specific mesenchymal lineages was not found. Many tumor cells contained densely eosinophilic hyaline globules in the cytoplasm. Tumor cells showed a loss of the nuclear expression of H3K27me3. Although cerebral sarcomas showing similar histopathological features have not been previously reported, a markedly myxoid change of the stroma admixed with delicate collagen fibers and "pseudolipoblasts" suggests a close pathological kinship to myxofibrosarcoma. Another notable finding was the appearance of many hyaline globules, which has been considered a characteristic finding of primary intracranial sarcoma, DICER1-mutant. In the cytogenetic study of the present case, however, no DICER1 gene mutations were demonstrated.