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Difference between revisions of "Subependymal giant cell astrocytoma"
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Subependymal giant cell astrocytoma (view source)
Revision as of 14:55, 8 October 2015
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = SEGA HE.jpg | |||
| Width = | |||
| Caption = Subependymal giant cell astrocytoma [[H&E stain]]. | |||
| Synonyms = SEGA | |||
| Micro = | |||
| Subtypes = | |||
| LMDDx = [[ganglioglioma]], [[pleomorphic xanthoastrocytoma]], [[glioblastoma]] | |||
| Stains = | |||
| IHC = GFAP +ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = brain - usu. wall of ventricles | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = rare - esp. in young adults | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = good (WHO Grade I) | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Subependymal giant cell astrocytoma''', abbreviated '''SEGA''', is a low-grade astrocytoma associated with [[tuberous sclerosis complex]]. | '''Subependymal giant cell astrocytoma''', abbreviated '''SEGA''', is a low-grade astrocytoma associated with [[tuberous sclerosis complex]]. | ||
==General== | ==General== | ||
*Associated with [[tuberous sclerosis complex]] (TSC).<ref name=pmid21455842>{{Cite journal | last1 = Grajkowska | first1 = W. | last2 = Kotulska | first2 = K. | last3 = Jurkiewicz | first3 = E. | last4 = Roszkowski | first4 = M. | last5 = Daszkiewicz | first5 = P. | last6 = Jóźwiak | first6 = S. | last7 = Matyja | first7 = E. | title = Subependymal giant cell astrocytomas with atypical histological features mimicking malignant gliomas. | journal = Folia Neuropathol | volume = 49 | issue = 1 | pages = 39-46 | month = | year = 2011 | doi = | PMID = 21455842 }}</ref> | *Associated with [[tuberous sclerosis complex]] (TSC).<ref name=pmid21455842>{{Cite journal | last1 = Grajkowska | first1 = W. | last2 = Kotulska | first2 = K. | last3 = Jurkiewicz | first3 = E. | last4 = Roszkowski | first4 = M. | last5 = Daszkiewicz | first5 = P. | last6 = Jóźwiak | first6 = S. | last7 = Matyja | first7 = E. | title = Subependymal giant cell astrocytomas with atypical histological features mimicking malignant gliomas. | journal = Folia Neuropathol | volume = 49 | issue = 1 | pages = 39-46 | month = | year = 2011 | doi = | PMID = 21455842 }}</ref> | ||
** 6-14% of all TSC patients will develop a SEGA. | |||
*Associated with epilepsy. | |||
*WHO Grade I. | *WHO Grade I. | ||
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*Well-demarcated. | *Well-demarcated. | ||
*Often projecting into a ventricle. | *Often projecting into a ventricle. | ||
*May be calcified | |||
*Circumscribed tumour. | |||
<gallery> | <gallery> | ||
| Line 16: | Line 52: | ||
==Microscopic== | ==Microscopic== | ||
Features:<ref name=upmc_case179/><ref name=pmid9595853>{{Cite journal | last1 = Taraszewska | first1 = A. | last2 = Kroh | first2 = H. | last3 = Majchrowski | first3 = A. | title = Subependymal giant cell astrocytoma: clinical, histologic and immunohistochemical characteristic of 3 cases. | journal = Folia Neuropathol | volume = 35 | issue = 3 | pages = 181-6 | month = | year = 1997 | doi = | PMID = 9595853 }}</ref> | Features:<ref name=upmc_case179/><ref name=pmid9595853>{{Cite journal | last1 = Taraszewska | first1 = A. | last2 = Kroh | first2 = H. | last3 = Majchrowski | first3 = A. | title = Subependymal giant cell astrocytoma: clinical, histologic and immunohistochemical characteristic of 3 cases. | journal = Folia Neuropathol | volume = 35 | issue = 3 | pages = 181-6 | month = | year = 1997 | doi = | PMID = 9595853 }}</ref> | ||
*Giant cells with nuclear atypia ("bizarre cells"). | *Giant cells with nuclear atypia ("bizarre cells", "ganglioid cells"). | ||
**[[Vesicular nuclei]]. | **[[Vesicular nuclei]]. | ||
**[[Nuclear pseudoinclusions]].<ref name=upmc179>URL: [http://path.upmc.edu/cases/case179/micro.html http://path.upmc.edu/cases/case179/micro.html]. Accessed on: 8 January 2012.</ref> | **[[Nuclear pseudoinclusions]].<ref name=upmc179>URL: [http://path.upmc.edu/cases/case179/micro.html http://path.upmc.edu/cases/case179/micro.html]. Accessed on: 8 January 2012.</ref> | ||
*Glassy eosinophilic cytoplasm. | *Glassy eosinophilic cytoplasm. | ||
*Elongated cells in a fibrillary background. | |||
*Abundant [[mast cell]]s.<ref name=upmc179>URL: [http://path.upmc.edu/cases/case179/micro.html http://path.upmc.edu/cases/case179/micro.html]. Accessed on: 8 January 2012.</ref> | *Abundant [[mast cell]]s.<ref name=upmc179>URL: [http://path.upmc.edu/cases/case179/micro.html http://path.upmc.edu/cases/case179/micro.html]. Accessed on: 8 January 2012.</ref> | ||
*Lymphocytic infiltrates. | |||
*Endothelial proliferations and/or necrosis are not a sign of malignancy. | |||
===Images=== | ===Images=== | ||
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*Vimentin +ve. (100%) | *Vimentin +ve. (100%) | ||
*S100 +ve. (100%) | *S100 +ve. (100%) | ||
* [[TTF-1]] (7 out of 7).<ref name=pmid25669749>{{Cite journal | last1 = Hewer | first1 = E. | last2 = Vajtai | first2 = I. | title = Consistent nuclear expression of thyroid transcription factor 1 in subependymal giant cell astrocytomas suggests lineage-restricted histogenesis. | journal = Clin Neuropathol | volume = 34 | issue = 3 | pages = 128-31 | month = | year = | doi = 10.5414/NP300818 | PMID = 25669749 }}</ref> | *Neurofilament +/-ve (ganglionic component). | ||
*Synaptophysin +/-ve (ganglionic component).. | |||
*[[TTF-1]] (7 out of 7).<ref name=pmid25669749>{{Cite journal | last1 = Hewer | first1 = E. | last2 = Vajtai | first2 = I. | title = Consistent nuclear expression of thyroid transcription factor 1 in subependymal giant cell astrocytomas suggests lineage-restricted histogenesis. | journal = Clin Neuropathol | volume = 34 | issue = 3 | pages = 128-31 | month = | year = | doi = 10.5414/NP300818 | PMID = 25669749 }}</ref> | |||
* MIB-1 usu. low (1-5%). | |||
==See also== | ==See also== | ||
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[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology tumours]] | [[Category:Neuropathology tumours]] | ||
[[Category:WHO grade I tumours]] | |||