CNS tumor
Ependymal tumors
Myxopapillary ependymoma
Author: Eman Abdelzaher, M.D., Ph.D.
Topic Completed: 1 August 2012
Minor changes: 19 November 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed search: Myxopapillary ependymoma [title]
Table of Contents
Definition / general | Epidemiology | Sites | Grading | Radiology description | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Differential diagnosisCite this page: Abdelzaher E. Myxopapillary ependymoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumormyxopapillaryependymoma.html. Accessed January 24th, 2021.
Definition / general
- Slow growing ependymal glioma composed of cells arranged in papillary pattern around vascularized myxoid cores
- May bleed into CSF
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
Grading
- Grade I of IV (WHO)
Radiology description
- Neuroimaging:
- Sharply circumscribed enhancing lesion (Radiographics 2006;26:S111)
- May show cystic change or hemorrhage
Case reports
- 33 year old man with back pain and pelvic mass (Case #399)
- 48 year old woman with back pain (Arch Pathol Lab Med 2004;128:811)
- 71 year old man with hypertension and low back pain (Case #111)
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
Case #111
On telfa pad
Microscopic (histologic) 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
Microscopic (histologic) images
Case #399
H&E (20x)
Diff-Quik (left: 20x, right: 40x)
PAP (left: 40x, right: 60x)
Brachyury (20x)
Cytokeratin AE1 / AE3 (20x)
Cytokeratin OSCAR (20x)
GFAP (20x)
S100 (20x)
Case #111
GFAP
Cytology description
- Nests and aggregates of epithelioid cells surrounding hyaline globules and branching cords of myxohyaline material
Negative stains
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-
