Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome

Tarik Tihan, Paul G. Fisher, James L. Kepner, Catherine Godfraind, Rodney D. McComb, Patricia T. Goldthwaite, Peter C. Burger

Research output: Contribution to journalArticle

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 regional. 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.

Original languageEnglish (US)
Pages (from-to)1061-1068
Number of pages8
JournalJournal of neuropathology and experimental neurology
Volume58
Issue number10
DOIs
StatePublished - Oct 1999

Keywords

  • Astrocytoma
  • Brain tumor
  • Glioma
  • Pediatric
  • Pilocytic
  • Pilomyxoid

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Fingerprint Dive into the research topics of 'Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome'. Together they form a unique fingerprint.

  • Cite this

    Tihan, T., Fisher, P. G., Kepner, J. L., Godfraind, C., McComb, R. D., Goldthwaite, P. T., & Burger, P. C. (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