Protoplasmic astrocytoma: Difference between revisions

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==Overview==
==Overview==
Protoplasmic astrocytoma is a rare variant of [[diffuse astrocytoma|diffuse low grade astrocytoma]] with characteristic histological and imaging features. It has been suggested that protoplasmic astrocytoma represents a variant of [[dysembryoplastic neuroepithelial tumor]]s (DNET), as they share histologic and imaging features. Currently, protoplasmic astrocytoma is classified as a subtype of diffuse low-grade astrocytoma.<ref name=overviewpa1>Protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016</ref>
Protoplasmic astrocytoma is a rare variant of [[diffuse astrocytoma|diffuse low grade astrocytoma]] with characteristic histological and imaging features. It has been suggested that protoplasmic astrocytoma represents a variant of [[dysembryoplastic neuroepithelial tumor]]s (DNET), as they share histologic and imaging features. Currently, protoplasmic astrocytoma is classified as a subtype of diffuse low-grade astrocytoma.
 
==Pathophysiology==
==Pathophysiology==
===Gross Pathology===
===Gross Pathology===
*Protoplasmic astrocytoma appear to have a predilection for the [[frontal lobe|frontal]] and [[temporal lobes]].<ref name=pathoilogypa1>Pathology of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016</ref>
*Protoplasmic astrocytoma appear to have a predilection for the [[frontal lobe|frontal]] and [[temporal lobes]].


===Microscopic Pathology===
===Microscopic Pathology===
*On microscopic histopathological analysis, protoplasmic astrocytoma is characterized by:<ref name=pathoilogypa1>Pathology of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016</ref><ref name=pathologyga1>Pathology of low grade infiltrative astrocytoma. Dr Ahmed Abd Rabou and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/low-grade-infiltrative-astrocytoma. Accessed on January 8, 2016</ref>
*On microscopic histopathological analysis, protoplasmic astrocytoma is characterized by:<ref name=pathoilogypa1>Pathology of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016
:*Neoplastic protoplasmic astrocytes
::*Scant cytoplasm
::*Scant cytoplasm
::*Rounded prominent nuclear contour
::*Rounded prominent nuclear contour
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===Immunohistochemistry===
===Immunohistochemistry===
*Protoplasmic astrocytoma is demonstrated by positivity to tumor marker such as [[GFAP]].<ref name=pathoilogypa1>Pathology of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016</ref>
*Protoplasmic astrocytoma is demonstrated by positivity to tumor marker such as [[GFAP]].


==Differentiating Protoplasmic Astrocytoma from other Diseases==
==Differentiating Protoplasmic Astrocytoma from other Diseases==
*Protoplasmic astrocytoma must be differentiated from:<ref name=ddxfa1>Differential diagnosis of low grade infiltrative astrocytoma. Dr Ahmed Abd Rabou and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/low-grade-infiltrative-astrocytoma. Accessed on January 5, 2016</ref>
*Protoplasmic astrocytoma must be differentiated from:
:*[[Fibrillary astrocytoma]]
:*[[Gemistocytic astrocytoma]]
:*[[Gemistocytic astrocytoma]]
:*[[Oligoastrocytoma]]
:*[[Oligoastrocytoma]]
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==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Age===
===Age===
*Protoplasmic astrocytoma is a rare disease that tends to affect young adults.<ref name=epidemiologypa1>Epidemiology of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016</ref>
*Protoplasmic astrocytoma is a rare disease that tends to affect young adults.
*The mean age at diagnosis is 32 years.
*The mean age at diagnosis is 32 years.


===Gender===
===Gender===
*Males are more commonly affected with protoplasmic astrocytoma than females. The male to female ratio is approximately 1.67 to 1.<ref name=epidemiologypa1>Epidemiology of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016</ref>
*Males are more commonly affected with protoplasmic astrocytoma than females. The male to female ratio is approximately 1.67 to 1.


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==

Revision as of 17:09, 15 July 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Synonyms and keywords: Protoplasmic astrocytomas; Diffuse astrocytoma; Low grade astrocytoma

Overview

Protoplasmic astrocytoma is a rare variant of diffuse low grade astrocytoma with characteristic histological and imaging features. It has been suggested that protoplasmic astrocytoma represents a variant of dysembryoplastic neuroepithelial tumors (DNET), as they share histologic and imaging features. Currently, protoplasmic astrocytoma is classified as a subtype of diffuse low-grade astrocytoma.

Pathophysiology

Gross Pathology

Microscopic Pathology

  • On microscopic histopathological analysis, protoplasmic astrocytoma is characterized by:
  • Protoplasmic astrocytoma is a slow growing tumor with an indolent course.

Complications

  • Common complication of protoplasmic astrocytoma include:[1]

History and Symptoms

History

  • When evaluating a patient for protoplasmic astrocytoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review.

Symptoms

  • Symptoms of protoplasmic astrocytoma include:[1]

CT

  • Head CT scan is helpful in the diagnosis of protoplasmic astrocytoma. On CT scan, protoplasmic astrocytoma is characterized by:[2]
  • Hypodense mass
  • Positive mass effect
  • No enhancement
  • Cystic or fluid attenuation, due to the aforementioned prominent mucinous microcystic component

MRI

  • Brain MRI is helpful in the diagnosis of protoplasmic astrocytoma. On MRI, protoplasmic astrocytoma is characterized by:[2][3]
MRI component Findings

T1

  • Hypointense compared to white matter

T2

  • Hyperintense compared to white matter

Fluid-attenuated inversion recovery (FLAIR)

  • Large areas of T2 hyperintensity suppress on FLAIR
  • These are not macrocystic, but rather represent the areas with abundant microcystic change

T1 with contrast

  • Little or no enhancement

Diffusion weighted imaging (DWI)

  • No restricted diffusion

Other Imaging Findings

Magnetic Resonance Spectroscopy

Magnetic Resonance Perfusion

  • MR perfusion may be helpful in the diagnosis of protoplasmic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).[2]

Electroencephalogram

  • Electroencephalogram (EEG) is performed in cases of protoplasmic astrocytoma to record the continuous electrical activity of the brain and locate the seizure activity.[4]

Biopsy

  • Biopsy of the protoplasmic astrocytoma tumor, taken through a needle during a simple surgical procedure, helps to confirm the diagnosis.[5]

Treatment

  • The treatment of protoplasmic astrocytoma depends on the clinical presentation, tumor size, and location.[5]
  • Surgery: The predominant therapy for protoplasmic astrocytoma is surgical resection.[5]
  • Radiotherapy: Radiotherapy may be used in protoplasmic astrocytoma post-operatively or at the time of recurrence or progression.[6]
  • Chemotherapy: Chemotherapy may have a role in recurrent and de-differentiated tumors.[6]


 
 
 
 
 
 
 
 
 
 
 
 
Treatment of protoplasmic astrocytoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Surgery
 
 
 
Radiotherapy
 
 
 
Chemotherapy
 
 
 
 
 
 
 
 
 
 
 
 
 

References

  1. ↑ 1.0 1.1 Clinical presentation of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016
  2. ↑ 2.0 2.1 2.2 2.3 Radiological features of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016
  3. ↑ Radiographic features of low grade infiltrative astrocytoma. Dr Ahmed Abd Rabou and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/low-grade-infiltrative-astrocytoma. Accessed on January 8, 2016
  4. ↑ Radiographic features of fibrillary astrocytoma. Dr Henry Knipe and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/fibrillary-astrocytoma. Accessed on January 4, 2016
  5. ↑ 5.0 5.1 5.2 Treatment and prognosis of protoplasmic astrocytoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/protoplasmic-astrocytoma. Accessed on January 8, 2016
  6. ↑ 6.0 6.1 Treatment and prognosis of low grade infiltrative astrocytoma. Dr Ahmed Abd Rabou and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/low-grade-infiltrative-astrocytoma. Accessed on January 8, 2016

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