Pleomorphic xanthoastrocytoma: Difference between revisions
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*The median age at diagnosis is 12 years.<ref name=agepxa2>Occurence of pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015</ref> | *The median age at diagnosis is 12 years.<ref name=agepxa2>Occurence of pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015</ref> | ||
==CT== | |||
Head CT scan is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On CT scan, pleomorphic xanthoastrocytoma is characterized by:<ref name=radpxa1>Radiographic features of pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015</ref> | |||
*Iso- or hypoattenuating mass | |||
*[[Calcification]] | |||
*Vivid enhancement | |||
==MRI== | ==MRI== | ||
Brain MRI is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On MRI, pleomorphic xanthoastrocytoma is characterized by:<ref name= | Brain MRI is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On MRI, pleomorphic xanthoastrocytoma is characterized by:<ref name=radpxa1>Radiographic features of pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015</ref> | ||
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Revision as of 19:49, 29 December 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Synonyms and keywords: Pleomorphic xanthoastrocytomas; PXA
Overview
Pleomorphic xanthoastrocytoma is a type of rare, low-grade astrocytoma (WHO Grade II) found in young patients who typically present with temporal lobe epilepsy.[1]
Pathophysiology
Pathogenesis
- Pleomorphic xanthoastrocytoma is formed through the mitosis of the astrocytes.[2]
Gross Pathology
- On gross pathology, polymorphic xanthoastrocytoma is characterized by a well circumscribed mass, often with cystic component and a superficially situated mural nodule, with involvement of the overlying leptomeninges.[3]
- Polymorphic xanthoastrocytomas are almost invariably (98%) located supratentorially, typically located superficially (peripherally), involving the cortex and overlying leptomeninges. Approximately half are located in the temporal lobe.
- They are found commonly in the area of the temples, frontal lobe, or on top of the parietal lobe. In about 20% of cases, more than one lobe is involved.[4]
Microscopic Pathology
- Ill-defined margins
- Spindle cells, polygonal cells, multi-nucleated cells, and lipid laden xanthomatous astrocytes
- Pleomorphic nuclei
- No endothelial proliferation or necrosis
Immunohistochemistry
Differentiating Pleomorphic Xanthoastrocytoma from other Diseases
Pleomorphic xanthoastrocytoma must be differentiated from:[5][6]
- Desmoplastic infantile ganglioglioma
- Neuroblastoma
- Extraventricular neurocytoma
- Dysembryoplastic neuroepithelial tumor (DNET)
- Ganglioglioma
- Oligodendroglioma
Epidemiology and Demographics
Prevalence
- Pleomorphic xanthoastrocytoma constitutes approximately 1% of all primary brain tumors.[7]
Age
- Pleomorphic xanthoastrocytoma is a rare disease that tends to affect the children and young adult population.[5]
- The median age at diagnosis is 12 years.[8]
CT
Head CT scan is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On CT scan, pleomorphic xanthoastrocytoma is characterized by:[9]
- Iso- or hypoattenuating mass
- Calcification
- Vivid enhancement
MRI
Brain MRI is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On MRI, pleomorphic xanthoastrocytoma is characterized by:[9]
| MRI component | Findings |
|---|---|
|
T1 |
|
|
T2 |
|
|
T1 with contrast |
|
References
- ↑ Pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015
- ↑ Pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015
- ↑ 3.0 3.1 3.2 Pathology of pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015
- ↑ Pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015
- ↑ 5.0 5.1 5.2 Yano, Hirohito; Saigoh, Chiemi; Nakayama, Noriyuki; Hirose, Yoshinobu; Abe, Masato; Ohe, Naoyuki; Ozeki, Michio; Shinoda, Jun; Iwama, Toru (2013). "Mixed neuronal-glial tumor in the temporal lobe of an infant: a case report". Diagnostic Pathology. 8 (1): 164. doi:10.1186/1746-1596-8-164. ISSN 1746-1596.
- ↑ Differential diagnosis of pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015
- ↑ Epidemiology of pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015
- ↑ Occurence of pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015
- ↑ 9.0 9.1 Radiographic features of pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on December 29, 2015