Difference between revisions of "Myxopapillary ependymoma"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = File:Myxopapillary_ependymoma_-_very_high_mag.jpg
| Image      = Myxopapillary_ependymoma_-_very_high_mag.jpg
| Width      =
| Width      =
| Caption    = Myxopapillary ependymoma. [[H&E stain]]
| Caption    = Myxopapillary ependymoma. [[HPS stain]].
| Synonyms  =
| Synonyms  =
| Micro      = Papillary tumor cells around vascular myxoid matrix
| Micro      = Papillary tumor cells around vascular myxoid matrix
| Subtypes  =
| Subtypes  = subtype of [[ependymoma]]
| LMDDx      = [[Ependymoma]
| LMDDx      = [[chordoma]], [[myxoid chondrosarcoma]], [[paraganglioma]], papillary [[adenocarcinoma]]
| Stains    = Alcian blue +ve  
| Stains    = Alcian blue +ve  
| IHC        = GFAP +ve
| IHC        = GFAP +ve
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| Tx        =
| Tx        =
}}
}}
'''Mxyopapillary Ependymoma''', is a low-grade [[Ependymoma]]. It is nearly always associated with [[cauda equina]] and [[filum terminale]].
'''Mxyopapillary Ependymoma''', is a low-grade [[Ependymoma]]. It is nearly always associated with [[cauda equina]] and [[filum terminale]].


==General==
==General==
*Low-grade [[Ependymoma]] - ''WHO Grade I'' by definition.
*Low-grade [[ependymoma]] - ''WHO Grade I'' by definition.
*Classically in the spinal cord of adults.  
*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>
* 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>
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==Gross==
==Gross==
*soft.
*Soft.
*gray.
*Gray.
*discrete masses.  
*Discrete masses.  
*often encapsulated.
*Often encapsulated.
*Subtotal resected tumors may spread throughout the neuraxis.
*Subtotal resected tumors may spread throughout the neuraxis.


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</gallery>
</gallery>


==Histology==
==Microscopic==
Features:
Features:
*Papillary appearance.
*Papillary appearance.
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*Microcysts.
*Microcysts.
*Low mitotic activity.
*Low mitotic activity.


Note:
Note:
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DDx:
DDx:
* [[Chordoma]]
* [[Chordoma]].
* [[Myxoid chondrosarcoma]]
* [[Myxoid chondrosarcoma]].
* [[Paraganglioma]]
* [[Paraganglioma]].
* Papillary [[Adenocarcinoma]]
* Papillary [[adenocarcinoma]].
Images:


===Images===
<gallery>
<gallery>
File:Myxopapillary_ependymoma.jpg | Mxyopapillary Ependymoma, HE Smear (AFIP)
File:Myxopapillary_ependymoma.jpg | Mxyopapillary Ependymoma, HE Smear (AFIP)
File:Myxopapillary_ependymoma_-_intermed_mag.jpg  | Mxyopapillary Ependymoma, low magnification (WC/Nephron)
File:Myxopapillary_ependymoma_-_intermed_mag.jpg  | Mxyopapillary Ependymoma, low magnification (WC/Nephron)
File:Myxopapillary_ependymoma-HE.jpg | Intermediate magnification (WC/jensflorian
File:Myxopapillary_ependymoma-HE.jpg | Intermediate magnification (WC/jensflorian)
File:Myxopapillary_ependymoma_-_very_high_mag.jpg | High magnification (WC/Nephron)
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_HE_x40.jpg | Mxyopapillary Ependymoma, vascular sclerosis (WC/jensflorian)
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==Molecular==
==Molecular==
* Poorly characterized
* Poorly characterized.
* No consistent abberations.
* No consistent abberations.


==See also==
==See also==

Latest revision as of 04:11, 2 May 2015

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.