Difference between revisions of "Myxopapillary ependymoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Myxopapillary_ependymoma_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Myxopapillary ependymoma. [[HPS stain]]. | |||
| Synonyms = | |||
| Micro = Papillary tumor cells around vascular myxoid matrix | |||
| Subtypes = subtype of [[ependymoma]] | |||
| LMDDx = [[chordoma]], [[myxoid chondrosarcoma]], [[paraganglioma]], papillary [[adenocarcinoma]] | |||
| 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> | |||
==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. <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]] |
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:
- Chordoma.
- Myxoid chondrosarcoma.
- Paraganglioma.
- Papillary adenocarcinoma.
Images
- Myxopapillary ependymoma - high mag. (WC).
- Myxopapillary ependymoma (bmj.com) - part of careers.bmj.com article on paediatric pathology.
- Myxopapillary ependymoma - cytology (WC).
- Myxopapillary ependymoma - several images (upmc.edu).
IHC
- GFAP+ve.
- S-100+ve.
- MIB-1 <1%.
Molecular
- Poorly characterized.
- No consistent abberations.
See also
References
- ↑ 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.
- ↑ 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.