Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome
(1999) Journal of Neuropathology and Experimental Neurology — Vol. 58, n° 10, p. 1061-1068 (1999)
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- Abstract
- Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA). The majority of the tumors occurred in infants and young children and involved the hypothalamic/chiasmatic region. The tumors were histologically similar to PA, but they were more monomorphous and more myxoid. Rosenthal fibers were not seen and only 1 of 18 tumors had eosinophilic granular bodies. At the end of the follow-up period, 6 patients were dead and 12 were alive with evidence of disease. Progression free survival (PFS) at 1 year was 38.7%. In comparison, we identified a control group of 13 classical PAs in the same age range and location as the study group. In this group, PFS at 1 year was 69.2%, which was significantly better than that for pilomyxoid tumors (p = 0.04). There was no CSF dissemination or death due to tumor progression among patients with classical PA. Eight of these patients are alive with recurrent disease, and 4 have no evidence of disease. While the monomorphous pilomyxoid tumors have some resemblance to classical PA, our results suggest that the former is a more aggressive variant or a separate entity that needs to be recognized for prognostic purposes.
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Tihan, T., Godfraind, C., Fisher, P., Kepner, J., McComb, R., Goldthwaite, P., & Burger, P. (1999). Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. Journal of Neuropathology and Experimental Neurology, 58(10), 1061-1068. https://doi.org/10.1097/00005072-199910000-00004 (Original work published 1999)