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 among young patients who typically present with temporal lobe epilepsy.[1]
Pathophysiology
Pathogenesis
- Pleomorphic xanthoastrocytoma is formed through the abnormal mitosis of the astrocytes.[2]
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
- 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]
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard |
Additional findings | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | |||||||||
| Lab Findings | MRI | Immunohistopathology | ||||||||
| Head- ache |
Seizure | Visual disturbance | Constitutional | Focal neurological deficit | ||||||
| Adult primary brain tumors | ||||||||||
| pleomorphic-xanthoastrocytoma |
+ | + | +/β | β | + | β |
|
|
| |
| Oligodendrocytoma | + | + | +/β | β | + | β |
|
|
| |
| Glioblastoma multiforme [7][8][9] |
+ | +/β | +/β | β | + | β |
|
|
| |
| Meningioma [10][11][12] |
+ | +/β | +/β | β | + | β |
|
|
| |
| Hemangioblastoma [13][14][15][16] |
+ | +/β | +/β | β | + | β |
|
| ||
| Pituitary adenoma [17][18][9] |
β | β | + Bitemporal hemianopia | β | β |
|
|
|
| |
| Schwannoma [19][20][21][22] |
β | β | β | β | + | β |
|
|
| |
| Primary CNS lymphoma [23][24] |
+ | +/β | +/β | β | + | β |
|
|
| |
| Childhood primary brain tumors | ||||||||||
| Pilocytic astrocytoma [25][26][27] |
+ | +/β | +/β | β | + | β |
|
|
| |
| Medulloblastoma [28][29][30] |
+ | +/β | +/β | β | + | β |
|
|
| |
| Ependymoma [31][9] |
+ | +/β | +/β | β | + | β |
|
|
| |
| Craniopharyngioma [32][33][34][9] |
+ | +/β | + Bitemporal hemianopia | β | + |
|
|
|
| |
| Pinealoma [35][36][37] |
+ | +/β | +/β | β | + vertical gaze palsy |
|
|
|
| |
| Vascular | ||||||||||
| AV malformation [38][39][9] |
+ | + | +/β | β | +/β | β |
|
| ||
| Brain aneurysm [40][41][42][43][44] |
+ | +/β | +/β | β | +/β | β |
|
|
|
|
| Infectious | ||||||||||
| Bacterial brain abscess [45][46] |
+ | +/β | +/β | + | + |
|
|
|
|
|
| Tuberculosis [47][9][48] |
+ | +/β | +/β | + | + |
|
|
|
|
|
| Toxoplasmosis [49][50] |
+ | +/β | +/β | β | + |
|
|
|
|
|
| Hydatid cyst [51][9] |
+ | +/β | +/β | +/β | + |
|
|
|
|
|
| CNS cryptococcosis [52] |
+ | +/β | +/β | + | + |
|
|
|
|
|
| CNS aspergillosis [53] |
+ | +/β | +/β | + | + |
|
|
|
|
|
| Other | ||||||||||
| Brain metastasis [54][9] |
+ | +/β | +/β | + | + | β |
|
|
|
|
ABBREVIATIONS
CNS=Central nervous system, AV=Arteriovenous, CSF=Cerebrospinal fluid, NF-2=Neurofibromatosis type 2, MEN-1=Multiple endocrine neoplasia, GFAP=Glial fibrillary acidic protein, HIV=Human immunodeficiency virus, BhCG=Human chorionic gonadotropin, ESR=Erythrocyte sedimentation rate, AFB=Acid fast bacilli, MRA=Magnetic resonance angiography, CTA=CT angiography
Epidemiology and Demographics
Prevalence
- Pleomorphic xanthoastrocytoma constitutes approximately 1% of all primary brain tumors.[55]
Age
- Pleomorphic xanthoastrocytoma is a rare disease that tends to affect children and young adults.[5]
- The median age at diagnosis is 26 years.[56]
Natural History, Complications and Prognosis
Natural History
If left untreated, patients with pleomorphic xanthoastrocytoma may progress to develop seizures, focal neurological deficits, and malignant transformation to either WHO grade III lesion or glioblastoma multiforme.[57]
Complication
Common complications of pleomorphic xanthoastrocytoma include:[57]
- Local recurrence
- Malignant transformation (WHO grade III astrocytoma or glioblastoma multiforme)
Prognosis
Prognosis is generally good, and the 5-year survival rate of patients with pleomorphic xanthoastrocytoma is approximately 70-80%.[57]
Symptoms
Symptoms of fibrillary astrocytoma include:[58][59]
- Elevated intracranial pressure
- Temporal lobe epilepsy
- Seizures with or without aura
- Amnesia
- Loss of consciousness
- Abnormal auditory sensations (sound or tune)
- Abnormal gustatory sensation (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:[60]
- 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:[60]
| MRI Component | Findings |
|---|---|
|
T1 weighted image |
|
|
T2 weighted image |
|
|
T1 weighted image with contrast |
|
Biopsy
Biopsy of pleomorphic xanthoastrocytoma tumor, taken through a needle during a simple surgical procedure, helps to confirm the diagnosis.[61]
Other Diagnostic Studies
Electroencephalogram
- Electroencephalogram (EEG) is performed among patients of pleomorphic xanthoastrocytoma to record any abnormal electrical activities (seizures).[59]
Digital Subtraction Angiography
- Digital subtraction angiography (DSA): Despite vivid enhancement, pleomorphic xanthoastrocytomas are usually avascular on angiography.[60]
Positron Emission Tomography
- F-18 FDG PET images of pleomorphic xanthoastrocytoma demonstrate markedly decreased accumulation of [18F]-fluorodeoxyglucose (glucose hypometabolism).[62]
Treatment
- The predominant therapy for pleomorphic xanthoastrocytoma is surgical resection. Adjunctive radiation may be indicated in cases of incompletely resected or recurrent tumors.[63][64]
- Complete surgical resection is often possible. However, the best choice of treatment will depend on many individual factors including:[63]
- 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.
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- β MartΓnez, RamΓ³n; 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. doi:10.1186/1471-2407-14-213. ISSNΒ 1471-2407.
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- β 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
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- β 60.0 60.1 60.2 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
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- β 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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