Primary Cerebral Pleomorphic-Myxoid Sarcoma With Many Hyaline Globules.
Masayuki Shintaku, Tetsuo Hashiba, Masahiro Nonaka et al.
✦ AI-curated · Sources linked
Case in brief
A 59-year-old man presented with a primary sarcoma in the right temporal lobe, following a history of pituitary tumor resection and radiotherapy 40 years prior. Histological examination revealed pleomorphic cells in a myxoid matrix with collagen fibers and pseudolipoblasts. Notably, many tumor cells contained eosinophilic hyaline globules. The tumor exhibited loss of H3K27me3 expression, and cytogenetic analysis showed no DICER1 mutations.
What made this case unusual
The presence of numerous eosinophilic hyaline globules in the tumor cells, typically associated with DICER1-mutant primary intracranial sarcomas, was unexpected given the absence of DICER1 mutations in this case.
Diagnostic / clinical pearl
Clinicians should consider the possibility of primary cerebral sarcomas with atypical histological features, such as hyaline globules, even in the absence of known genetic mutations like DICER1.
Why it matters
This case highlights the potential for misinterpretation of histological features that may suggest specific genetic mutations, emphasizing the need for thorough cytogenetic evaluation in atypical sarcomas.