Difference between revisions of "Myxopapillary ependymoma"

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#redirect [[Ependymoma#Myxopapillary_ependymoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = File:Myxopapillary_ependymoma_-_very_high_mag.jpg
| Width      =
| Caption    = Myxopapillary ependymoma. [[H&E stain]]
| Synonyms  =
| Micro      = Papillary tumor cells around vascular myxoid matrix
| Subtypes  =
| LMDDx      = [[Ependymoma]
| Stains    = Alcian blue +ve
| IHC        = GFAP +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = usually lumbar spinal cord
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good (WHO Grade I)
| Other      =
| ClinDDx    =
| Tx        =
}}
 
'''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.<ref>{{Cite journal  | last1 = Schiffer | first1 = D. | last2 = Chiò | first2 = A. | last3 = Giordana | first3 = MT. | last4 = Migheli | first4 = A. | last5 = Palma | first5 = L. | last6 = Pollo | first6 = B. | last7 = Soffietti | first7 = R. | last8 = Tribolo | first8 = A. | title = Histologic prognostic factors in ependymoma. | journal = Childs Nerv Syst | volume = 7 | issue = 4 | pages = 177-82 | month = Aug | year = 1991 | doi =  | PMID = 1933913 }}</ref>
*Associated with back pain.
*Enhancing mass
 
==Gross==
*soft.
*gray.
*discrete masses.
*often encapsulated.
*Subtotal resected tumors may spread throughout the neuraxis.
 
<gallery>
File:AFIP405714G-MYXOPAPILLARY EPENDYMOMA.jpg | Myxopapillary ependymoma in the filum terminale (Dr. E. Michael Scott, Boston, MA).
File:Myxopapillary_ependymoma_1.jpg | Mxyopapillary Ependymoma in the spinal cord, cross section (AFIP)
</gallery>
 
==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. <ref>{{Cite journal  | last1 = Schittenhelm | first1 = J. | last2 = Becker | first2 = R. | last3 = Capper | first3 = D. | last4 = Meyermann | first4 = R. | last5 = Iglesias-Rozas | first5 = JR. | last6 = Kaminsky | first6 = J. | last7 = Mittelbronn | first7 = M. | title = The clinico-surgico-pathological spectrum of myxopapillary ependymomas--report of four unusal cases and review of the literature. | journal = Clin Neuropathol | volume = 27 | issue = 1 | pages = 21-8 | month =  | year =  | doi =  | PMID = 18257471 }}</ref>
 
DDx:
* [[Chordoma]]
* [[Myxoid chondrosarcoma]]
* [[Paraganglioma]]
* Papillary [[Adenocarcinoma]]
Images:
 
<gallery>
File:Myxopapillary_ependymoma.jpg | Mxyopapillary Ependymoma, HE Smear (AFIP)
File:Myxopapillary_ependymoma_-_intermed_mag.jpg  | Mxyopapillary Ependymoma, low magnification (WC/Nephron)
File:Myxopapillary_ependymoma-HE.jpg | Intermediate magnification (WC/jensflorian
File:Myxopapillary_ependymoma_-_very_high_mag.jpg | High magnification (WC/Nephron)
File:Myxopapillary_ependymoma_HE_x40.jpg | Mxyopapillary Ependymoma, vascular sclerosis (WC/jensflorian)
File:Myxopapillary_Ependymoma_Alcian-blue.jpg | Alcian-blue staining highlighting the mucinous matrix (WC/jensflorian)
</gallery>
*[http://commons.wikimedia.org/wiki/File:Myxopapillary_ependymoma_-_high_mag.jpg Myxopapillary ependymoma - high mag. (WC)].
*[http://careers.bmj.com/article-images/cf0708.f2_default.gif Myxopapillary ependymoma (bmj.com)] - part of [http://careers.bmj.com/careers/advice/view-article.html?id=351 careers.bmj.com article on paediatric pathology].
*[http://commons.wikimedia.org/wiki/File:Myxopapillary_ependymoma.jpg Myxopapillary ependymoma - cytology (WC)].
*[http://path.upmc.edu/cases/case626.html Myxopapillary ependymoma - several images (upmc.edu)].
 
==IHC==
* GFAP+ve.
* S-100+ve.
* MIB-1 <1%.
 
==Molecular==
* Poorly characterized
* No consistent abberations.
 
 
==See also==
*[[Neuropathology tumours]].
*[[Ependymoma]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Neuropathology tumours]]

Revision as of 14:36, 27 April 2015

{{ Infobox diagnosis | Name = Myxopapillary ependymoma | Image = File:Myxopapillary_ependymoma_-_very_high_mag.jpg | Width = | Caption = Myxopapillary ependymoma. H&E stain | Synonyms = | Micro = Papillary tumor cells around vascular myxoid matrix | Subtypes = | LMDDx = [[Ependymoma] | Stains = Alcian blue +ve | IHC = GFAP +ve | EM = | Molecular = | IF = | Gross = | Grossing = | Site = usually lumbar spinal cord | Assdx = | Syndromes = | Clinicalhx = | Signs = | Symptoms = | Prevalence = | Bloodwork = | Rads = | Endoscopy = | Prognosis = good (WHO Grade I) | Other = | ClinDDx = | Tx = }}

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.

  • Myxopapillary ependymoma in the filum terminale (Dr. E. Michael Scott, Boston, MA).

  • Mxyopapillary Ependymoma in the spinal cord, cross section (AFIP)

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:

  • Mxyopapillary Ependymoma, HE Smear (AFIP)

  • Mxyopapillary Ependymoma, low magnification (WC/Nephron)

  • Intermediate magnification (WC/jensflorian

  • High magnification (WC/Nephron)

  • Mxyopapillary Ependymoma, vascular sclerosis (WC/jensflorian)

  • Alcian-blue staining highlighting the mucinous matrix (WC/jensflorian)

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.
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