Pleomorphic xanthoastrocytoma
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
Gross Pathology
- On gross pathology, polymorphic xanthoastrocytoma is characterized by a well circumscribed mass, often with cystic component and involvement of the overlying leptomeninges.[2]
- 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.
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:[3][4]
- 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.[5]
Age
- Pleomorphic xanthoastrocytoma is a rare disease that tends to affect the children and young adult population.[3]
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
- ↑ 2.0 2.1 2.2 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
- ↑ 3.0 3.1 3.2 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