Microglioma in a child - A further case in support of the microglioma entity and distinction from histiocytic sarcoma

Anitha Mathews, Keiko Ohsawa, Michael E. Buckland, Chandrasekharan Kesavadas, Kuttan Ratheesan, Parukuttyamma Kusumakumary, Peter C. Burger, Shinichi Kohsaka, Manuel B. Graeber

Research output: Contribution to journalArticlepeer-review

Abstract

Microglia are not generally known to cause brain tumors but one bona fide case of adult microglioma has been published [9]. This tumor was highly malignant. We now report on a second, juvenile case, which showed a less aggressive course. Microglioma is a primary central nervous system (CNS) neoplasm distinct from glioma and other known brain tumor entities, based on its strong immunoreactivity for the macrophage marker CD163, the microglia marker Iba1, and the complete absence of neural as well as lymphocyte antigens. Furthermore, we have analyzed the literature and identified a number of cases that qualify as primary parenchymal histiocytic sarcomas of the CNS, which lack microglial morphology. Considering the non-hematopoietic developmental origin of the vast majority of microglia and the distinct morphological as well as immunophenotypic similarity of their neoplastic counterparts, we suggest using the term microglioma. More cases will be required along with appropriately-collected tissue to establish the molecular genetic profile of this extremely rare entity.

Original languageEnglish (US)
Pages (from-to)302-313
Number of pages12
JournalClinical neuropathology
Volume35
Issue number5
DOIs
StatePublished - 2016

Keywords

  • Gliomatosis cerebri
  • Microglia
  • Monocytes
  • Yolk sac
  • glioma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology

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