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

238 Citations (Scopus)

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

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Astrocytoma
Pediatrics
Neoplasms
Disease-Free Survival
Hospital Pathology Department
Recurrence
Control Groups

Keywords

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

ASJC Scopus subject areas

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

Cite this

Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. / Tihan, Tarik; Fisher, Paul G.; Kepner, James L.; Godfraind, Catherine; McComb, Rodney D; Goldthwaite, Patricia T.; Burger, Peter C.

In: Journal of Neuropathology and Experimental Neurology, Vol. 58, No. 10, 10.1999, p. 1061-1068.

Research output: Contribution to journalArticle

Tihan, Tarik ; Fisher, Paul G. ; Kepner, James L. ; Godfraind, Catherine ; McComb, Rodney D ; Goldthwaite, Patricia T. ; Burger, Peter C. / Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. In: Journal of Neuropathology and Experimental Neurology. 1999 ; Vol. 58, No. 10. pp. 1061-1068.
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