Myxopapillary ependymoma

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Myxopapillary ependymoma
Diagnosis in short

Myxopapillary ependymoma. HPS stain.

LM Papillary tumor cells around vascular myxoid matrix
Subtypes subtype of ependymoma
LM DDx chordoma, myxoid chondrosarcoma, paraganglioma, papillary adenocarcinoma
Stains Alcian blue +ve
IHC GFAP +ve
Site usually lumbar spinal cord

Prognosis good (WHO Grade I)

Mxyopapillary Ependymoma, is a low-grade Ependymoma. It is nearly always associated with cauda equina and filum terminale.

General

  • Low-grade ependymoma - WHO Grade I by definition.
  • Classically in the spinal cord of adults.
  • Approx 9-13% of all ependymal tumors.[1]
  • Associated with back pain.
  • Enhancing mass

Gross

  • Soft.
  • Gray.
  • Discrete masses.
  • Often encapsulated.
  • Subtotal resected tumors may spread throughout the neuraxis.

Microscopic

Features:

  • Papillary appearance.
  • Perivascular pseudorosettes:
    • Cuboidal to elongated tumor cells.
    • Radially arranged around vascular cores.
  • Myxoid material surround blood vessels.
  • Microcysts.
  • Low mitotic activity.

Note: Cases with extensive sclerosis may mimic degenerative changes. [2]

DDx:

Images

IHC

  • GFAP+ve.
  • S-100+ve.
  • MIB-1 <1%.

Molecular

  • Poorly characterized.
  • No consistent abberations.

See also

References

  1. Schiffer, D.; Chiò, A.; Giordana, MT.; Migheli, A.; Palma, L.; Pollo, B.; Soffietti, R.; Tribolo, A. (Aug 1991). "Histologic prognostic factors in ependymoma.". Childs Nerv Syst 7 (4): 177-82. PMID 1933913.
  2. Schittenhelm, J.; Becker, R.; Capper, D.; Meyermann, R.; Iglesias-Rozas, JR.; Kaminsky, J.; Mittelbronn, M.. "The clinico-surgico-pathological spectrum of myxopapillary ependymomas--report of four unusal cases and review of the literature.". Clin Neuropathol 27 (1): 21-8. PMID 18257471.