Myxopapillary ependymoma

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Myxopapillary ependymoma
Diagnosis in short

Myxopapillary ependymoma. H&E stain

LM Papillary tumor cells around vascular myxoid matrix
LM DDx Ependymoma
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.

Histology

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.