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==Positron Emission Tomography==
==Positron Emission Tomography==
*[[PET|F-18 FDG PET]] images of pleomorphic xanthoastrocytoma demonstrates markedly decreased accumulation of [[Florbetapir (18F)|[18F]-fluorodeoxyglucose]] ([[glucose metabolism|glucose hypometabolism]]).
*[[PET|F-18 FDG PET]] images of pleomorphic xanthoastrocytoma demonstrates markedly decreased accumulation of [[Florbetapir (18F)|[18F]-fluorodeoxyglucose]] ([[glucose metabolism|glucose hypometabolism]]).<ref name="pmid15015766">{{cite journal| author=Im SH, Chung CK, Kim SK, Cho BK, Kim MK, Chi JG| title=Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes. | journal=J Neurooncol | year= 2004 | volume= 66 | issue= 1-2 | pages= 17-27 | pmid=15015766 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15015766  }} </ref>


==References==
==References==

Revision as of 20:00, 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

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

  • On microscopic histopathological analysis, polymorphic xanthoastrocytoma is characterized by:[3][5]
  • Ill-defined margins
  • Spindle cells, polygonal cells, multi-nucleated cells, and lipid laden xanthomatous astrocytes
  • Pleomorphic nuclei
  • No endothelial proliferation or necrosis

Immunohistochemistry

  • Polymorphic xanthoastrocytoma is demonstrated by positivity to tumor marker such as GFAP.[3]

Differentiating Pleomorphic Xanthoastrocytoma from other Diseases

Pleomorphic xanthoastrocytoma must be differentiated from:[5][6]

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 on contrast administration

MRI

Brain MRI is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On MRI, pleomorphic xanthoastrocytoma is characterized by:[9]

MRI component Findings

T1

  • Solid component: iso- to hypointense with respect to the gray matter
  • Cystic component: hypointense
  • Leptomeningeal involvement

T2

  • Solid component: iso- to hyperintense with respect to the gray matter
  • Cystic component: hyperintense
  • Mild surrounding vasogenic edema

T1 with contrast

  • Solid component: vivid enhancement

Positron Emission Tomography

References

  1. 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. Pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015
  3. 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
  4. Pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015
  5. 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.
  6. 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
  7. 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
  8. Occurence of pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on December 29, 2015
  9. 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
  10. Im SH, Chung CK, Kim SK, Cho BK, Kim MK, Chi JG (2004). "Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes". J Neurooncol. 66 (1–2): 17–27. PMID 15015766.

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