CNS tumor
Ependymal tumors
Myxopapillary ependymoma

Authors: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 30 August 2016, last major update August 2012

Copyright: (c) 2005-2016, PathologyOutlines.com, Inc.

PubMed search: myxopapillary ependymoma[title]
Cite this page: Myxopapillary ependymoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumormyxopapillaryependymoma.html. Accessed December 2nd, 2016.
Definition / General
  • Slow growing ependymal glioma composed of cells arranged in papillary pattern around vascularized myxoid cores
  • May bleed into CSF
Grading
  • Grade I of IV (WHO)
Epidemiology
  • 2/3 male, mean age 36 years
Sites
  • Almost exclusively near conus medullaris, cauda equina, filum terminale of spinal cord
  • Occasionally cervical-thoracic spine, intraventricular, brain parenchyma
  • May arise as subcutaneous sacrococcygeal or presacral mass
Radiology Description
Radiology Images

Images hosted on other servers:

Intradural extramedullary mass

Case Reports
Treatment
  • Gross total resection may be most predictive factor for outcome (Clin Neuropathol 2008;27:21)
  • Incomplete removal may cause local or widespread dissemination and distant metastases
Gross Description
  • Encapsulated lobulated soft masses that are easily resected
Gross Images

Images hosted on other servers:

On telfa pad

Micro Description
  • Well differentiated cuboidal to elongated tumor cells radially oriented around vascularized myxoid cores with a myxopapillary appearance
  • Usually no atypia, no / low mitotic activity
Micro Images

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Images hosted on PathOut server:

Case of the Week #399:

H&E (20x)

DiffQuik (left: 20x, right: 40x)

PAP (left: 40x, right: 60x)


Brachyury (20x)

Cytokeratin AE1/AE3 (20x)

Cytokeratin OSCAR (20x)

GFAP (20x)

S100 (20x)



Case of the Week #111:

GFAP



Images hosted on other servers:

Papillary structures with core of acellular hyaline material

Papillary tumor with rosette-like structures surrounding mucoid area with central vessels

Subcutaneous sacrococcygeal tumor



Contributed by Dr. Ankur Sangoi, El Camino Hospital, California (USA):

Frozen sections


Squash preps


Permanent sections


Permanent sections

Alcian Blue

EMA

Cytology Description
  • Nests and aggregates of epithelioid cells surrounding hyaline globules and branching cords of myxohyaline material
Positive Stains
  • GFAP, mucin (PAS or Alcian blue, including vessel walls), vimentin, S100 (50%)
Negative Stains
  • Cytokeratin
Electron Microscopy Description
  • Basal bodies and cilia are present but may be difficult to find
  • Resembles choroid plexus with cells containing nonciliated intracytoplasmic lumina or abnormal arrays of microtubules
Differential Diagnosis
  • Carcinoma: keratin+, GFAP-
  • Chordoma: cords and lobules of physaliphorous cells, keratin+, GFAP-
  • Meningioma: mucin-, GFAP-
  • Myxoid chondrosarcoma
  • Paraganglioma: cells are more uniform and epithelial and rest on capillary walls, salt and pepper chromatin; secretory granules (EM), mucin-, GFAP-
  • Schwannoma: mucin-