Difference between revisions of "Subependymoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Subependymoma_-_very_high_mag.jpg
| Width      =
| Caption    = Subependymoma. [[H&E stain]].
| Synonyms  =
| Micro      = microcysts with bluish material (give a spongy appearance at low magnification), clustering of nuclei cluster (described as "bundles of flowers"), bland nuclei
| Subtypes  =
| LMDDx      =
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[brain]] - see ''[[neuropathology tumours]]''
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  = +/-headache
| Prevalence =
| Bloodwork  =
| Rads      = classically fourth ventricle
| Endoscopy  =
| Prognosis  = WHO grade I
| Other      =
| ClinDDx    = other brain tumours - [[ependymoma]], [[CNS lymphoma]]
| Tx        = surgical excision
}}
'''Subependymoma''' is [[neuropathology]] tumour classically found in the fourth ventricle.
'''Subependymoma''' is [[neuropathology]] tumour classically found in the fourth ventricle.


==General==
==General==
*Good prognosis - WHO Grade I.
*Good prognosis - WHO Grade I (ICD-O 9383/1).
*Low-grade glial tumour of the middle-aged and elderly.<ref name=pmid23483468/>
*Formerly called ''subependymal (glomerate) astrocytoma''
 
Clinical:<ref name=pmid23483468>{{Cite journal  | last1 = Castro-Castro | first1 = J. | last2 = Castro-Bouzas | first2 = D. | last3 = Prieto-Casal | first3 = PL. | last4 = Carcacia-Hermilla | first4 = ID. | last5 = Riu-Lloveras | first5 = M. | last6 = Castro-Gómez | first6 = JE. | title = [Subependymoma of the lateral ventricle. A case report]. | journal = Rev Neurol | volume = 56 | issue = 6 | pages = 332-6 | month = Mar | year = 2013 | doi =  | PMID = 23483468 }}
</ref>
*Slow growing.
*+/-Headaches.
*CSF obstructions.
*Tx: surgery.


==Gross/radiology==
==Gross/radiology==
*Classic location: fourth ventricle.<ref>{{Cite journal  | last1 = Hoeffel | first1 = C. | last2 = Boukobza | first2 = M. | last3 = Polivka | first3 = M. | last4 = Lot | first4 = G. | last5 = Guichard | first5 = JP. | last6 = Lafitte | first6 = F. | last7 = Reizine | first7 = D. | last8 = Merland | first8 = JJ. | title = MR manifestations of subependymomas. | journal = AJNR Am J Neuroradiol | volume = 16 | issue = 10 | pages = 2121-9 | month =  | year =  | doi =  | PMID = 8585504 |url=http://www.ajnr.org/cgi/reprint/16/10/2121}}</ref>
*Classic location: fourth ventricle (50-60%).<ref>{{Cite journal  | last1 = Hoeffel | first1 = C. | last2 = Boukobza | first2 = M. | last3 = Polivka | first3 = M. | last4 = Lot | first4 = G. | last5 = Guichard | first5 = JP. | last6 = Lafitte | first6 = F. | last7 = Reizine | first7 = D. | last8 = Merland | first8 = JJ. | title = MR manifestations of subependymomas. | journal = AJNR Am J Neuroradiol | volume = 16 | issue = 10 | pages = 2121-9 | month =  | year =  | doi =  | PMID = 8585504 |url=http://www.ajnr.org/cgi/reprint/16/10/2121}}</ref>
*Well demarcated margin.
*Lateral ventricles (30-40% of all cases), rarely IIIrd ventricle, septum pellucideum and spinal cord
*Well-demarcated margin.
*Usu. completely within the ventricle; does not extend into brain (like [[ependymoma]]s).
*Usu. completely within the ventricle; does not extend into brain (like [[ependymoma]]s).
*May be hemorrhagic. <ref>{{Cite journal  | last1 = Landriel | first1 = F. | last2 = Besada | first2 = C. | last3 = Migliaro | first3 = M. | last4 = Christiansen | first4 = S. | last5 = Goldschmidt | first5 = E. | last6 = Yampolsky | first6 = C. | last7 = Ajler | first7 = P. | title = Atypical hemorrhagic presentation of a fourth ventricle subependymoma: case report. | journal = Neurol Med Chir (Tokyo) | volume = 53 | issue = 11 | pages = 828-31 | month =  | year = 2013 | doi =  | PMID = 24140775 }}</ref>
*Usually less than 2cm in diameter.
*Incidentally found at autopsies.
===Images===
<gallery>
File:AFIP405740R-SUBEPENDYMOMA.jpg | Subependymoma (AFIP)
File:Subependymoma.jpg | Macroscopy (AFIP)
</gallery>


==Microscopic==
==Microscopic==
Features:<ref name=ouhsc>URL: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm]. Accessed on: 2 June 2011.</ref>
Features:<ref name=ouhsc>URL: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm]. Accessed on: 2 June 2011.</ref>
*Microcysts with bluish material - give a spongy appearance at low magnification.
*Microcysts with bluish material - give a spongy appearance at low magnification.
*Nuclei cluster.
*Isomorphic nuclei cluster embedded into fibrillary matrix.
**Described as "bundles of flowers".
**Described as "bundles of flowers".
*Calcifications possible
*Combined subependymomas/classical cellular ependymomas (then grade II)


Negatives.
Negatives.
*No nuclear pleomorphism, no prominent nucleoli, no mitoses.
*No nuclear pleomorphism, no prominent nucleoli, no mitoses.
*Do not invade into brain.<ref name=pmid23483468/>


===Images===
===Images===
www:
www: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com05Image/Com501-2-04.gif Subependymoma (ouhsc.edu)].<ref name=ouhsc>URL: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm]. Accessed on: 2 June 2011.</ref>
*[http://moon.ouhsc.edu/kfung/jty1/Com05/Com05Image/Com501-2-04.gif Subependymoma (ouhsc.edu)].<ref name=ouhsc>URL: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm]. Accessed on: 2 June 2011.</ref>
<gallery>
<gallery>
File:Subependymoma HE x40.jpg | Subependymoma - low mag. (WC/jensflorian)
Image:Subependymoma_-_intermed_mag.jpg | Subependyoma - intermed. mag. (WC)
Image:Subependymoma_-_intermed_mag.jpg | Subependyoma - intermed. mag. (WC)
Image:Subependymoma_-_high_mag.jpg | Subependymoma - high mag. (WC)
Image:Subependymoma_-_high_mag.jpg | Subependymoma - high mag. (WC)
File:Subependymoma 2 - high mag.jpg | Subependymoma - high mag. (WC/Nephron)
Image:Subependymoma_-_very_high_mag.jpg | Subependymoma - very high mag. (WC)
File:AFIP405743M-SUBEPENDYMOMA.jpg | Subependymoma (AFIP)
</gallery>
</gallery>
DDx:
[[Ependymoma]]
==IHC==
*GFAP+ve
*MIB-1 very low (1%)
*ATRX: no loss
*IDH1(R132H)-ve
==Molecular==
*Posterior fossa and spine: chr.6 alterations.
*Supratentorial: No consistent abberations.


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

Latest revision as of 14:00, 8 July 2020

Subependymoma
Diagnosis in short

Subependymoma. H&E stain.
LM microcysts with bluish material (give a spongy appearance at low magnification), clustering of nuclei cluster (described as "bundles of flowers"), bland nuclei
Site brain - see neuropathology tumours
Symptoms +/-headache
Radiology classically fourth ventricle
Prognosis WHO grade I
Clin. DDx other brain tumours - ependymoma, CNS lymphoma
Treatment surgical excision

Subependymoma is neuropathology tumour classically found in the fourth ventricle.

General

  • Good prognosis - WHO Grade I (ICD-O 9383/1).
  • Low-grade glial tumour of the middle-aged and elderly.[1]
  • Formerly called subependymal (glomerate) astrocytoma

Clinical:[1]

  • Slow growing.
  • +/-Headaches.
  • CSF obstructions.
  • Tx: surgery.

Gross/radiology

  • Classic location: fourth ventricle (50-60%).[2]
  • Lateral ventricles (30-40% of all cases), rarely IIIrd ventricle, septum pellucideum and spinal cord
  • Well-demarcated margin.
  • Usu. completely within the ventricle; does not extend into brain (like ependymomas).
  • May be hemorrhagic. [3]
  • Usually less than 2cm in diameter.
  • Incidentally found at autopsies.

Images

  • Subependymoma (AFIP)

  • Macroscopy (AFIP)

Microscopic

Features:[4]

  • Microcysts with bluish material - give a spongy appearance at low magnification.
  • Isomorphic nuclei cluster embedded into fibrillary matrix.
    • Described as "bundles of flowers".
  • Calcifications possible
  • Combined subependymomas/classical cellular ependymomas (then grade II)

Negatives.

  • No nuclear pleomorphism, no prominent nucleoli, no mitoses.
  • Do not invade into brain.[1]

Images

www: Subependymoma (ouhsc.edu).[4]

  • Subependymoma - low mag. (WC/jensflorian)

  • Subependyoma - intermed. mag. (WC)

  • Subependymoma - high mag. (WC)

  • Subependymoma - high mag. (WC/Nephron)

  • Subependymoma - very high mag. (WC)

  • Subependymoma (AFIP)

DDx: Ependymoma

IHC

  • GFAP+ve
  • MIB-1 very low (1%)
  • ATRX: no loss
  • IDH1(R132H)-ve

Molecular

  • Posterior fossa and spine: chr.6 alterations.
  • Supratentorial: No consistent abberations.

See also

References

  1. ↑ 1.0 1.1 1.2 Castro-Castro, J.; Castro-Bouzas, D.; Prieto-Casal, PL.; Carcacia-Hermilla, ID.; Riu-Lloveras, M.; Castro-Gómez, JE. (Mar 2013). "[Subependymoma of the lateral ventricle. A case report].". Rev Neurol 56 (6): 332-6. PMID 23483468.
  2. ↑ Hoeffel, C.; Boukobza, M.; Polivka, M.; Lot, G.; Guichard, JP.; Lafitte, F.; Reizine, D.; Merland, JJ.. "MR manifestations of subependymomas.". AJNR Am J Neuroradiol 16 (10): 2121-9. PMID 8585504. http://www.ajnr.org/cgi/reprint/16/10/2121.
  3. ↑ Landriel, F.; Besada, C.; Migliaro, M.; Christiansen, S.; Goldschmidt, E.; Yampolsky, C.; Ajler, P. (2013). "Atypical hemorrhagic presentation of a fourth ventricle subependymoma: case report.". Neurol Med Chir (Tokyo) 53 (11): 828-31. PMID 24140775.
  4. ↑ 4.0 4.1 URL: http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm. Accessed on: 2 June 2011.
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