Pleomorphic xanthoastrocytoma

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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 among 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 a cystic component and a superficially situated mural nodule.[3]
  • Polymorphic xanthoastrocytomas are almost invariably (98%) located supratentorially, typically involving the cortex and overlying leptomeninges. Approximately half are located in the temporal lobe.
  • They are found commonly in the area of the temporal lobe, frontal lobe, or superior to 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

  • On immunohistochemistry, polymorphic xanthoastrocytoma is characterized by a positive GFAP tumor marker.[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 children and young adults.[5]
  • The median age at diagnosis is 26 years.[8]

Natural History, Complications and Prognosis

Natural History

If left untreated, patients with pleomorphic xanthoastrocytoma may progress to develop seizures, focal neurological deficits, and maliganant transformation to either WHO grade III lesion or glioblastoma multiforme.[9]

Complication

Common complications of pleomorphic xanthoastrocytoma include:[9]

Prognosis

Prognosis is generally good, and the 5-year survival rate of patients with pleomorphic xanthoastrocytoma is approximately 70-80%.[9]

Symptoms

Symptoms of fibrillary astrocytoma include:[10][11]

  • Elevated intracranial pressure
  • Temporal lobe epilepsy
  • Seizures with or without aura
  • Amnesia
  • Loss of consciousness
  • Abnormal auditory sensations (sound or tune)
  • Abnormal gustatory senasation (taste)
  • Abnormal olfactory sensation (smell that is not truly present)
  • Dysphoria or euphoria
  • Motionless staring, automatic movements of the hands or mouth, inability to respond to others, unusual speech, or unusual behavior
  • Neurological symptoms

CT

Head CT scan is helpful in the diagnosis of pleomorphic xanthoastrocytoma. On CT scan, pleomorphic xanthoastrocytoma is characterized by:[12]

  • 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:[12]

MRI Component Findings

T1 weighted image

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

T2 weighted image

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

T1 weighted image with contrast

  • Solid component: vivid enhancement

Biopsy

Biopsy of pleomorphic xanthastrocytoma tumor, taken through a needle during a simple surgical procedure, helps to confirm the diagnosis.[13]

Other Diagnostic Studies

Electroencephalogram

  • Electroencephalogram (EEG) is performed among patients of pleomorphic xanthoastrocytoma to record any abnormal electrical activities (seizures).[11]

Digital Subtraction Angiography

Positron Emission Tomography

Treatment

  • The predominant therapy for pleomorphic xanthoastrocytoma is surgical resection. Adjunctive radiation may be indicated in cases of incompletely resected or recurrent tumors.[15][16]
  • Complete surgical resection is often possible. However, the best choice of treatment will depend on many individual factors including:[15]
  • Patient's medical history and overall health condition
  • Type, location, and size of the tumor
  • Patient's age
  • Aggressiveness of the tumor
  • If surgery is performed and the tumor is completely resected, further treatment may not be required.

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. ISSN 1746-1596. doi:10.1186/1746-1596-8-164. 
  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. Martínez, Ramón; Carmona, F; Vizoso, Miguel; Rohde, Veit; Kirsch, Matthias; Schackert, Gabriele; Ropero, Santiago; Paulus, Werner; Barrantes, Alonso; Gomez, Antonio; Esteller, Manel (2014). "DNA methylation alterations in grade II- and anaplastic pleomorphic xanthoastrocytoma". BMC Cancer. 14 (1): 213. ISSN 1471-2407. doi:10.1186/1471-2407-14-213. 
  9. 9.0 9.1 9.2 Treatment and prognosis 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. Clinical presentation of pleomorphic xanthoastrocytoma. Pleomorphic xanthoastrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pleomorphic-xanthoastrocytoma. Accessed on January 4, 2015
  11. 11.0 11.1 Symptoms and diagnosis of pleomorphic xanthoastrocytoma. Dana-farber & Boston children cancer and blood disorders center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/pleomorphic-xanthoastrocytoma.aspx. Accessed on January 4, 2015
  12. 12.0 12.1 12.2 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
  13. Diagnosis of pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on January 4, 2015
  14. 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. 
  15. 15.0 15.1 Treatment of pleomorphic xanthoastrocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pleomorphic_xanthoastrocytoma. Accessed on January 4, 2015
  16. Progressive or recurrent disease of pleomorphic xanthoastrocytoma. Dana-farber & Boston children cancer and blood disorders center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/pleomorphic-xanthoastrocytoma.aspx. Accessed on January 4, 2015

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