CNS tumor
Embryonal tumors
Atypical teratoid / rhabdoid tumor

Author: Nat Pernick, M.D. (see Authors page)

Revised: 1 September 2017, last major update January 2006

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: "Atypical teratoid / rhabdoid tumor" CNS

Cite this page: Pernick, N. Atypical teratoid / rhabdoid tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumoratypicalteratoidrhabdoid.html. Accessed October 19th, 2017.
Definition / general
  • Not in WHO classification
  • Rare; first described in 1987
  • Infants and young children (mean age 17 months)
  • Usually posterior fossa or supratentorial
  • Very aggressive with poor prognosis (mean survival 11 months postsurgery); metastasizes throughout CSF
Radiology images

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Heterogenous mass

Case reports
Microscopic (histologic) description
  • Large and pleomorphic rhabdoid cells with abundant eosinophilic cytoplasm (pinker than PNET), often filamentous cytoplasmic inclusions and vacuoles
  • Eccentric round nuclei and prominent nucleolus
  • May have PNET-like areas with small cells
  • May have mucinous background resembling chordoma
  • May have epithelioid features with poorly formed glands or Flexner-Wintersteiner rosettes
Microscopic (histologic) images

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Various images

H&E

Epithelioid areas


Vimentin

EMA

GFAP

Spinal tumor

Cytology description
  • Hypercellular
  • Large tissue fragments with papillary appearance of large tumor cells surrounding capillaries
  • Cells are large, round and plasmacytoid or rhabdoid (intermediate size with granular to fibrillary, brightly eosinophilic cytoplasm and variable inclusions; large, eccentric nuclei with single prominent nucleolus)
  • Also small, round, primitive, neural type cells with high N/C ratio
  • Also apoptotic bodies, mitotic figures, marked necrosis
  • Variable dystrophic calcification, bizarre, multinucleated giant cells (Cancer 2005;105:65)
Cytology images

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Typical cytology

Negative stains
Electron microscopy description
  • Globular / fibrillar paranuclear inclusions (whorled intermediate filaments)
Molecular / cytogenetics description
Differential diagnosis