Protoplasmic astrocytoma: Difference between revisions

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__NOTOC__
__NOTOC__
{{CMG}}{{AE}}{{SR}}
{{CMG}}{{AE}}{{SR}}{{RAK}}


{{SK}} Protoplasmic astrocytomas; Diffuse astrocytoma; Low grade astrocytoma
{{SK}} Protoplasmic astrocytomas; Diffuse astrocytoma; Low grade astrocytoma


==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.
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. The possibility that a primary cerebral neoplasm represents a protoplasmic astrocytoma should be considered in a patient with a large frontal or temporal tumor that has a very high signal on T2 with a large proportion of the tumor showing substantial T2 FLAIR suppression.


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===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
*On microscopic histopathological analysis, protoplasmic astrocytoma is characterized by:<ref name="pmid7785654">{{cite journal| author=Prayson RA, Estes ML| title=Protoplasmic astrocytoma. A clinicopathologic study of 16 tumors. | journal=Am J Clin Pathol | year= 1995 | volume= 103 | issue= 6 | pages= 705-9 | pmid=7785654 | doi=10.1093/ajcp/103.6.705 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7785654  }} </ref>


::*Scant cytoplasm
::*Scant cytoplasm
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:*Mucinous fluid containing microcystic spaces (prominent feature)
:*Mucinous fluid containing microcystic spaces (prominent feature)
:*Abscence of [[mitoses]], microvascular proliferation, and [[necrosis]]
:*Abscence of [[mitoses]], microvascular proliferation, and [[necrosis]]
:*temporal and frontal lobes were the most likely site of origin.<nowiki><ref name="pmid7785654"></nowiki>{{cite journal| author=Prayson RA, Estes ML| title=Protoplasmic astrocytoma. A clinicopathologic study of 16 tumors. | journal=Am J Clin Pathol | year= 1995 | volume= 103 | issue= 6 | pages= 705-9 | pmid=7785654 | doi=10.1093/ajcp/103.6.705 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7785654  }}
:*temporal and frontal lobes were the most likely site of origin.
</ref>


===Immunohistochemistry===
===Immunohistochemistry===
*Protoplasmic astrocytoma is demonstrated by positivity to tumor marker such as [[GFAP]].
*Protoplasmic astrocytoma is demonstrated by positivity to tumor marker such as [[GFAP]].


== Causes ==
There are no causes for protoplasmic astrocytoma.
==Differentiating Protoplasmic Astrocytoma from other Diseases==
==Differentiating Protoplasmic Astrocytoma from other Diseases==
*Protoplasmic astrocytoma must be differentiated from:
*Protoplasmic astrocytoma must be differentiated from:
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===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="pmid7785654">{{cite journal| author=Prayson RA, Estes ML| title=Protoplasmic astrocytoma. A clinicopathologic study of 16 tumors. | journal=Am J Clin Pathol | year= 1995 | volume= 103 | issue= 6 | pages= 705-9 | pmid=7785654 | doi=10.1093/ajcp/103.6.705 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7785654  }} </ref>
*Males are more commonly affected with protoplasmic astrocytoma than females. The male to female ratio is approximately 1.67 to 1.<ref name="pmid7785654">{{cite journal| author=Prayson RA, Estes ML| title=Protoplasmic astrocytoma. A clinicopathologic study of 16 tumors. | journal=Am J Clin Pathol | year= 1995 | volume= 103 | issue= 6 | pages= 705-9 | pmid=7785654 | doi=10.1093/ajcp/103.6.705 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7785654  }} </ref>
== Risk Factors ==
There are no established risk factors for protoplasmic astrocytoma.
== Screening ==
There is insufficient evidence to recommend routine screening for protoplasmic astrocytoma.


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
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:*[[Hydrocephalus]]
:*[[Hydrocephalus]]


==History and Symptoms==
== Diagnosis ==
===History===
 
=== Diagnostic Study of Choice ===
The diagnosis of protoplasmic astrocytoma is based on a tissue biopsy.
 
===History and Symptoms===
 
*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.
*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:<ref name="symptomspa1">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</ref>
*Symptoms of protoplasmic astrocytoma include:<ref name="symptomspa1">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</ref>
:*[[Headaches]]
:*[[Headaches]]
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:*[[Speech difficulties]]
:*[[Speech difficulties]]


==CT==
=== Physical Examination ===
Patients with protoplasmic astrocytoma usually appear
 
=== Laboratory Findings ===
There are no diagnostic laboratory findings associated with protoplasmic astrocytoma.
 
=== Electrocardiogram ===
There are no ECG findings associated with protoplasmic astrocytoma.
 
=== X-ray ===
There are no x-ray findings associated with protoplasmic astrocytoma.
 
=== Echocardiography or Ultrasound ===
There are no echocardiography/ultrasound findings associated with protoplasmic astrocytoma.
 
===CT scan===
 
*Head CT scan is helpful in the diagnosis of protoplasmic astrocytoma. On CT scan, protoplasmic astrocytoma is characterized by:<ref name="radiologicalfeaturspa1">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</ref>
*Head CT scan is helpful in the diagnosis of protoplasmic astrocytoma. On CT scan, protoplasmic astrocytoma is characterized by:<ref name="radiologicalfeaturspa1">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</ref>
:*Hypodense mass  
:*Hypodense mass  
:*Positive mass effect
:*Positive mass effect
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:*Cystic or fluid attenuation, due to the aforementioned prominent mucinous microcystic component
:*Cystic or fluid attenuation, due to the aforementioned prominent mucinous microcystic component


==MRI==
===MRI===
 
*The possibility that a primary cerebral neoplasm represents a protoplasmic astrocytoma should be considered in a patient with a large frontal or temporal tumor that has a very high signal on T2 with a large proportion of the tumor showing substantial T2 FLAIR suppression.<ref name="pmid20644924">{{cite journal| author=Tay KL, Tsui A, Phal PM, Drummond KJ, Tress BM| title=MR imaging characteristics of protoplasmic astrocytomas. | journal=Neuroradiology | year= 2011 | volume= 53 | issue= 6 | pages= 405-11 | pmid=20644924 | doi=10.1007/s00234-010-0741-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20644924  }} </ref>
*The possibility that a primary cerebral neoplasm represents a protoplasmic astrocytoma should be considered in a patient with a large frontal or temporal tumor that has a very high signal on T2 with a large proportion of the tumor showing substantial T2 FLAIR suppression.<ref name="pmid20644924">{{cite journal| author=Tay KL, Tsui A, Phal PM, Drummond KJ, Tress BM| title=MR imaging characteristics of protoplasmic astrocytomas. | journal=Neuroradiology | year= 2011 | volume= 53 | issue= 6 | pages= 405-11 | pmid=20644924 | doi=10.1007/s00234-010-0741-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20644924  }} </ref>
*Brain MRI is helpful in the diagnosis of protoplasmic astrocytoma. On MRI, protoplasmic astrocytoma is characterized by:<ref name="radiologicalfeaturspa1">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</ref><ref name="radiographicfeaturesga1">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</ref>
*Brain MRI is helpful in the diagnosis of protoplasmic astrocytoma. On MRI, protoplasmic astrocytoma is characterized by:<ref name="radiologicalfeaturspa1">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</ref><ref name="radiographicfeaturesga1">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</ref>
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|}
|}


==Other Imaging Findings==
===Other Imaging Findings===
===Magnetic Resonance Spectroscopy===
====Magnetic Resonance Spectroscopy====
 
*[[Nuclear magnetic resonance spectroscopy|MR spectroscopy]] may be helpful in the diagnosis of protoplasmic astrocytoma, which demonstrates elevated [[choline]]/[[creatine]] ratio.<ref name="radiologicalfeaturspa1">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</ref>
*[[Nuclear magnetic resonance spectroscopy|MR spectroscopy]] may be helpful in the diagnosis of protoplasmic astrocytoma, which demonstrates elevated [[choline]]/[[creatine]] ratio.<ref name="radiologicalfeaturspa1">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</ref>


===Magnetic Resonance Perfusion===
====Magnetic Resonance Perfusion====
 
*[[Perfusion weighted imaging|MR perfusion]] may be helpful in the diagnosis of protoplasmic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).<ref name="radiologicalfeaturspa1">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</ref>
*[[Perfusion weighted imaging|MR perfusion]] may be helpful in the diagnosis of protoplasmic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).<ref name="radiologicalfeaturspa1">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</ref>


==Electroencephalogram==
<br />
 
=== Other Diagnostic Studies ===
 
====Electroencephalogram====
 
*[[Electroencephalogram|Electroencephalogram (EEG)]] is performed in cases of protoplasmic astrocytoma to record the continuous electrical activity of the brain and locate the seizure activity.<ref name="radfa1">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</ref>
*[[Electroencephalogram|Electroencephalogram (EEG)]] is performed in cases of protoplasmic astrocytoma to record the continuous electrical activity of the brain and locate the seizure activity.<ref name="radfa1">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</ref>


==Biopsy==
==Treatment==
*[[Biopsy]] of the protoplasmic astrocytoma tumor, taken through a needle during a simple surgical procedure, helps to confirm the diagnosis.<ref name="treatmentandprognosispa1">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</ref>
 
=== Medical Therapy ===
 
There is no specific chemotherapy regimen for protoplasmic astrocytoma. It is managed as an astrocytoma.
 
*Post surgical medical therapy is recommended in all patients with astrocytoma tumor.
 
===Astrocytoma===
 
*'''1 Grade 1 and 2 - Low grade astrocytomas'''
**1.1 Wait and see<ref name="pmid17469128">{{cite journal |vauthors=Ricard D, Kaloshi G, Amiel-Benouaich A, Lejeune J, Marie Y, Mandonnet E, Kujas M, Mokhtari K, Taillibert S, Laigle-Donadey F, Carpentier AF, Omuro A, Capelle L, Duffau H, Cornu P, Guillevin R, Sanson M, Hoang-Xuan K, Delattre JY |title=Dynamic history of low-grade gliomas before and after temozolomide treatment |journal=Ann. Neurol. |volume=61 |issue=5 |pages=484–90 |date=May 2007 |pmid=17469128 |doi=10.1002/ana.21125 |url=}}</ref><ref name="pmid18976072">{{cite journal |vauthors=Shaw EG, Berkey B, Coons SW, Bullard D, Brachman D, Buckner JC, Stelzer KJ, Barger GR, Brown PD, Gilbert MR, Mehta M |title=Recurrence following neurosurgeon-determined gross-total resection of adult supratentorial low-grade glioma: results of a prospective clinical trial |journal=J. Neurosurg. |volume=109 |issue=5 |pages=835–41 |date=November 2008 |pmid=18976072 |pmc=3833272 |doi=10.3171/JNS/2008/109/11/0835 |url=}}</ref>
***The wait and see approach is for young patient with complete or nearly complete [[tumor]] resection.
***since the [[low grade astrocytoma]] will finally grow to [[high grade astrocytoma]], we should screen these patients with contrast [[MRI]] every 4 month.
**1.2 [[Radiation therapy]]<ref name="pmid16168780">{{cite journal |vauthors=van den Bent MJ, Afra D, de Witte O, Ben Hassel M, Schraub S, Hoang-Xuan K, Malmström PO, Collette L, Piérart M, Mirimanoff R, Karim AB |title=Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial |journal=Lancet |volume=366 |issue=9490 |pages=985–90 |date=2005 |pmid=16168780 |doi=10.1016/S0140-6736(05)67070-5 |url=}}</ref><ref name="pmid26530266">{{cite journal |vauthors=Ryken TC, Parney I, Buatti J, Kalkanis SN, Olson JJ |title=The role of radiotherapy in the management of patients with diffuse low grade glioma: A systematic review and evidence-based clinical practice guideline |journal=J. Neurooncol. |volume=125 |issue=3 |pages=551–83 |date=December 2015 |pmid=26530266 |doi=10.1007/s11060-015-1948-1 |url=}}</ref>
*** Immediate post [[surgery]] [[radiation therapy]] can reduce the progression rate.
*** It doesn't affect survival since it cannot prevent transformation of [[low grade astrocytoma]] to [[high grade astrocytoma]].
***Preferred regimen: 50 t0 54 Gy
**1.3 Adjunctive [[chemotherapy]]<ref name="pmid27050206">{{cite journal |vauthors=Buckner JC, Shaw EG, Pugh SL, Chakravarti A, Gilbert MR, Barger GR, Coons S, Ricci P, Bullard D, Brown PD, Stelzer K, Brachman D, Suh JH, Schultz CJ, Bahary JP, Fisher BJ, Kim H, Murtha AD, Bell EH, Won M, Mehta MP, Curran WJ |title=Radiation plus Procarbazine, CCNU, and Vincristine in Low-Grade Glioma |journal=N. Engl. J. Med. |volume=374 |issue=14 |pages=1344–55 |date=April 2016 |pmid=27050206 |pmc=5170873 |doi=10.1056/NEJMoa1500925 |url=}}</ref><ref name="pmid22851558">{{cite journal |vauthors=Shaw EG, Wang M, Coons SW, Brachman DG, Buckner JC, Stelzer KJ, Barger GR, Brown PD, Gilbert MR, Mehta MP |title=Randomized trial of radiation therapy plus procarbazine, lomustine, and vincristine chemotherapy for supratentorial adult low-grade glioma: initial results of RTOG 9802 |journal=J. Clin. Oncol. |volume=30 |issue=25 |pages=3065–70 |date=September 2012 |pmid=22851558 |pmc=3732006 |doi=10.1200/JCO.2011.35.8598 |url=}}</ref>
***1.3.1 [[Temozolomide]]
***1.3.2 PVC ([[Procarbazine]], [[Lomustine]], [[Vincristine]])
***Based on previous studies, patients who get [[chemotherapy]] along with [[Radiation therapy|radiotherapy]] immediately after [[surgery]] has better outcome.


==Treatment==
<br />
*The treatment of protoplasmic astrocytoma depends on the clinical presentation, tumor size, and location.<ref name="treatmentandprognosispa1">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</ref>


:*'''Surgery''': The predominant therapy for protoplasmic astrocytoma is [[surgery|surgical resection]].<ref name="treatmentandprognosispa1">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</ref>
*'''2 Grade 3 - [[Anaplastic astrocytoma]]'''
:*'''Radiotherapy''': [[Radiotherapy]] may be used in protoplasmic astrocytoma post-operatively or at the time of recurrence or progression.<ref name="biopsyga1">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</ref>
**2.1 [[Radiation therapy|Radiotherapy]]+ [[chemotherapy]] ([[Temozolomide]])<ref name="pmid26033545">{{cite journal |vauthors=Juratli TA, Lautenschläger T, Geiger KD, Pinzer T, Krause M, Schackert G, Krex D |title=Radio-chemotherapy improves survival in IDH-mutant, 1p/19q non-codeleted secondary high-grade astrocytoma patients |journal=J. Neurooncol. |volume=124 |issue=2 |pages=197–205 |date=September 2015 |pmid=26033545 |doi=10.1007/s11060-015-1822-1 |url=}}</ref><ref name="pmid27401155">{{cite journal |vauthors=Shin JY, Diaz AZ |title=Anaplastic astrocytoma: prognostic factors and survival in 4807 patients with emphasis on receipt and impact of adjuvant therapy |journal=J. Neurooncol. |volume=129 |issue=3 |pages=557–565 |date=September 2016 |pmid=27401155 |doi=10.1007/s11060-016-2210-1 |url=}}</ref>
:*'''Chemotherapy''': [[Chemotherapy]] may have a role in recurrent and de-differentiated tumors.<ref name="biopsyga1">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</ref>
***Studies demonstrated that the combination of [[radiotherapy]] and [[chemotherapy]] with [[temozolomide]] is more effective.
**2.2 [[Radiation therapy|Radiation]]
** 2.3 [[Chemotherapy]]
***2.3.1 [[Procarbazine]], [[Lomustine]], [[Vincristine]]
***2.3.2 [[Procarbazine]], [[Lomustine]], [[Temozolomide]]<ref name="pmid19901110">{{cite journal |vauthors=Wick W, Hartmann C, Engel C, Stoffels M, Felsberg J, Stockhammer F, Sabel MC, Koeppen S, Ketter R, Meyermann R, Rapp M, Meisner C, Kortmann RD, Pietsch T, Wiestler OD, Ernemann U, Bamberg M, Reifenberger G, von Deimling A, Weller M |title=NOA-04 randomized phase III trial of sequential radiochemotherapy of anaplastic glioma with procarbazine, lomustine, and vincristine or temozolomide |journal=J. Clin. Oncol. |volume=27 |issue=35 |pages=5874–80 |date=December 2009 |pmid=19901110 |doi=10.1200/JCO.2009.23.6497 |url=}}</ref><ref name="pmid27370396">{{cite journal |vauthors=Wick W, Roth P, Hartmann C, Hau P, Nakamura M, Stockhammer F, Sabel MC, Wick A, Koeppen S, Ketter R, Vajkoczy P, Eyupoglu I, Kalff R, Pietsch T, Happold C, Galldiks N, Schmidt-Graf F, Bamberg M, Reifenberger G, Platten M, von Deimling A, Meisner C, Wiestler B, Weller M |title=Long-term analysis of the NOA-04 randomized phase III trial of sequential radiochemotherapy of anaplastic glioma with PCV or temozolomide |journal=Neuro-oncology |volume=18 |issue=11 |pages=1529–1537 |date=November 2016 |pmid=27370396 |pmc=5063521 |doi=10.1093/neuonc/now133 |url=}}</ref>


<br />


{{familytree/start |summary=Treatment of protoplasmic astrocytoma}}
*'''3 Grade 4 - [[Glioblastoma multiforme|Glioblastoma multiform]]'''
{{familytree |boxstyle=background: #DCDCDC;| | | | | | | | | | | | | A01| | | | | | | | | | | | | |A01=<div style="width: 12em; padding:0.2em;">'''Treatment of protoplasmic astrocytoma'''</div>}}
**3.1 [[Chemotherapy]] (± [[Radiation therapy|radiotherapy]])<ref name="pmid15758009">{{cite journal |vauthors=Stupp R, Mason WP, van den Bent MJ, Weller M, Fisher B, Taphoorn MJ, Belanger K, Brandes AA, Marosi C, Bogdahn U, Curschmann J, Janzer RC, Ludwin SK, Gorlia T, Allgeier A, Lacombe D, Cairncross JG, Eisenhauer E, Mirimanoff RO |title=Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma |journal=N. Engl. J. Med. |volume=352 |issue=10 |pages=987–96 |date=March 2005 |pmid=15758009 |doi=10.1056/NEJMoa043330 |url=}}</ref><ref name="pmid27172136">{{cite journal |vauthors=Kole AJ, Park HS, Yeboa DN, Rutter CE, Corso CD, Aneja S, Lester-Coll NH, Mancini BR, Knisely JP, Yu JB |title=Concurrent chemoradiotherapy versus radiotherapy alone for "biopsy-only" glioblastoma multiforme |journal=Cancer |volume=122 |issue=15 |pages=2364–70 |date=August 2016 |pmid=27172136 |doi=10.1002/cncr.30063 |url=}}</ref>
{{familytree |boxstyle=background: #DCDCDC;| | | | | | | |,|-|-|-|-|-|+|-|-|-|-|-|.| | | | | | | | | | | | | | |}}
***3.1.1 [[Temozolomide]]
{{familytree |boxstyle=background: #DCDCDC;| | | | | | | B01 | | | | B02 | | | | B03 | | | | | | | | | | | | | |B01=<div style="width: 9em; padding:0.2em;">'''Surgery''' </div>|B02=<div style="width: 9em; padding:0.2em;">'''Radiotherapy'''</div>|B03=<div style="width: 9em; padding:0.2em;">'''Chemotherapy'''</div>}}
**3.2 [[Bevacizumab]]<ref name="pmid21135282">{{cite journal |vauthors=Lai A, Tran A, Nghiemphu PL, Pope WB, Solis OE, Selch M, Filka E, Yong WH, Mischel PS, Liau LM, Phuphanich S, Black K, Peak S, Green RM, Spier CE, Kolevska T, Polikoff J, Fehrenbacher L, Elashoff R, Cloughesy T |title=Phase II study of bevacizumab plus temozolomide during and after radiation therapy for patients with newly diagnosed glioblastoma multiforme |journal=J. Clin. Oncol. |volume=29 |issue=2 |pages=142–8 |date=January 2011 |pmid=21135282 |pmc=3058273 |doi=10.1200/JCO.2010.30.2729 |url=}}</ref>
{{familytree/end}}
*** A [[Monoclonal antibodies|monoclonal antibody]] which bind to [[VEGF]] and inactivate it.
**3.3 Alternating electric fields<ref name="pmid22608262">{{cite journal |vauthors=Stupp R, Wong ET, Kanner AA, Steinberg D, Engelhard H, Heidecke V, Kirson ED, Taillibert S, Liebermann F, Dbalý V, Ram Z, Villano JL, Rainov N, Weinberg U, Schiff D, Kunschner L, Raizer J, Honnorat J, Sloan A, Malkin M, Landolfi JC, Payer F, Mehdorn M, Weil RJ, Pannullo SC, Westphal M, Smrcka M, Chin L, Kostron H, Hofer S, Bruce J, Cosgrove R, Paleologous N, Palti Y, Gutin PH |title=NovoTTF-100A versus physician's choice chemotherapy in recurrent glioblastoma: a randomised phase III trial of a novel treatment modality |journal=Eur. J. Cancer |volume=48 |issue=14 |pages=2192–202 |date=September 2012 |pmid=22608262 |doi=10.1016/j.ejca.2012.04.011 |url=}}</ref>
***A portable device which will be placed on the [[scalp]] for generating TT fields. The combination of this device with [[Temozolomide]] will significantly increase survival.
**3.4 [[Carmustine]] polymer wafers<ref name="pmid12672279">{{cite journal |vauthors=Westphal M, Hilt DC, Bortey E, Delavault P, Olivares R, Warnke PC, Whittle IR, Jääskeläinen J, Ram Z |title=A phase 3 trial of local chemotherapy with biodegradable carmustine (BCNU) wafers (Gliadel wafers) in patients with primary malignant glioma |journal=Neuro-oncology |volume=5 |issue=2 |pages=79–88 |date=April 2003 |pmid=12672279 |pmc=1920672 |doi=10.1093/neuonc/5.2.79 |url=}}</ref>
*** Implanted at the time of [[surgery]].
***Can be used in combination with [[chemotherapy]] or [[radiation]].


<br />
==References==
==References==
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{{reflist|2}}

Latest revision as of 22:11, 27 July 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]Roukoz A. Karam, M.D.[3]

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. The possibility that a primary cerebral neoplasm represents a protoplasmic astrocytoma should be considered in a patient with a large frontal or temporal tumor that has a very high signal on T2 with a large proportion of the tumor showing substantial T2 FLAIR suppression.


Classification

Protoplasmic astrocytoma is a subtype of astrocytoma and is included in the classification of astrocytoma. For more information about the classification of astrocytoma, click here.

Pathophysiology

Gross Pathology

Microscopic Pathology

  • On microscopic histopathological analysis, protoplasmic astrocytoma is characterized by:[1]
  • Scant cytoplasm
  • Rounded prominent nuclear contour
  • Flaccid processes
  • Low cellular density
  • Mild nuclear atypia (enlarged, irregular contour, hyperchromasia, and coarsened nuclear chromatin pattern)
  • Mucinous fluid containing microcystic spaces (prominent feature)
  • Abscence of mitoses, microvascular proliferation, and necrosis
  • temporal and frontal lobes were the most likely site of origin.

Immunohistochemistry

  • Protoplasmic astrocytoma is demonstrated by positivity to tumor marker such as GFAP.

Causes

There are no causes for protoplasmic astrocytoma.

Differentiating Protoplasmic Astrocytoma from other Diseases

  • Protoplasmic astrocytoma must be differentiated from:

Epidemiology and Demographics

Age

  • Protoplasmic astrocytoma is a rare disease that tends to affect young adults.
  • The mean age at diagnosis is 32 years.

Gender

  • Males are more commonly affected with protoplasmic astrocytoma than females. The male to female ratio is approximately 1.67 to 1.[1]

Risk Factors

There are no established risk factors for protoplasmic astrocytoma.

Screening

There is insufficient evidence to recommend routine screening for protoplasmic astrocytoma.

Natural History, Complications and Prognosis

Natural History

  • If left untreated, patients with protoplasmic astrocytoma may progress to develop seizures, focal neurological deficits, and hydrocephalus.[2]
  • Protoplasmic astrocytoma is a slow growing tumor with an indolent course.

Complications

  • Common complication of protoplasmic astrocytoma include:[2]

Diagnosis

Diagnostic Study of Choice

The diagnosis of protoplasmic astrocytoma is based on a tissue biopsy.

History and Symptoms

  • 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 of protoplasmic astrocytoma include:[2]

Physical Examination

Patients with protoplasmic astrocytoma usually appear

Laboratory Findings

There are no diagnostic laboratory findings associated with protoplasmic astrocytoma.

Electrocardiogram

There are no ECG findings associated with protoplasmic astrocytoma.

X-ray

There are no x-ray findings associated with protoplasmic astrocytoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with protoplasmic astrocytoma.

CT scan

  • Head CT scan is helpful in the diagnosis of protoplasmic astrocytoma. On CT scan, protoplasmic astrocytoma is characterized by:[3]
  • Hypodense mass
  • Positive mass effect
  • No enhancement
  • Cystic or fluid attenuation, due to the aforementioned prominent mucinous microcystic component

MRI

  • The possibility that a primary cerebral neoplasm represents a protoplasmic astrocytoma should be considered in a patient with a large frontal or temporal tumor that has a very high signal on T2 with a large proportion of the tumor showing substantial T2 FLAIR suppression.[4]
  • Brain MRI is helpful in the diagnosis of protoplasmic astrocytoma. On MRI, protoplasmic astrocytoma is characterized by:[3][5]
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).[3]


Other Diagnostic Studies

Electroencephalogram

  • Electroencephalogram (EEG) is performed in cases of protoplasmic astrocytoma to record the continuous electrical activity of the brain and locate the seizure activity.[6]

Treatment

Medical Therapy

There is no specific chemotherapy regimen for protoplasmic astrocytoma. It is managed as an astrocytoma.

  • Post surgical medical therapy is recommended in all patients with astrocytoma tumor.

Astrocytoma




References

  1. ↑ 1.0 1.1 Prayson RA, Estes ML (1995). "Protoplasmic astrocytoma. A clinicopathologic study of 16 tumors". Am J Clin Pathol. 103 (6): 705–9. doi:10.1093/ajcp/103.6.705. PMID 7785654.
  2. ↑ 2.0 2.1 2.2 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
  3. ↑ 3.0 3.1 3.2 3.3 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
  4. ↑ Tay KL, Tsui A, Phal PM, Drummond KJ, Tress BM (2011). "MR imaging characteristics of protoplasmic astrocytomas". Neuroradiology. 53 (6): 405–11. doi:10.1007/s00234-010-0741-2. PMID 20644924.
  5. ↑ 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
  6. ↑ 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
  7. ↑ Ricard D, Kaloshi G, Amiel-Benouaich A, Lejeune J, Marie Y, Mandonnet E, Kujas M, Mokhtari K, Taillibert S, Laigle-Donadey F, Carpentier AF, Omuro A, Capelle L, Duffau H, Cornu P, Guillevin R, Sanson M, Hoang-Xuan K, Delattre JY (May 2007). "Dynamic history of low-grade gliomas before and after temozolomide treatment". Ann. Neurol. 61 (5): 484–90. doi:10.1002/ana.21125. PMID 17469128.
  8. ↑ Shaw EG, Berkey B, Coons SW, Bullard D, Brachman D, Buckner JC, Stelzer KJ, Barger GR, Brown PD, Gilbert MR, Mehta M (November 2008). "Recurrence following neurosurgeon-determined gross-total resection of adult supratentorial low-grade glioma: results of a prospective clinical trial". J. Neurosurg. 109 (5): 835–41. doi:10.3171/JNS/2008/109/11/0835. PMC 3833272. PMID 18976072.
  9. ↑ van den Bent MJ, Afra D, de Witte O, Ben Hassel M, Schraub S, Hoang-Xuan K, Malmström PO, Collette L, Piérart M, Mirimanoff R, Karim AB (2005). "Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial". Lancet. 366 (9490): 985–90. doi:10.1016/S0140-6736(05)67070-5. PMID 16168780.
  10. ↑ Ryken TC, Parney I, Buatti J, Kalkanis SN, Olson JJ (December 2015). "The role of radiotherapy in the management of patients with diffuse low grade glioma: A systematic review and evidence-based clinical practice guideline". J. Neurooncol. 125 (3): 551–83. doi:10.1007/s11060-015-1948-1. PMID 26530266.
  11. ↑ Buckner JC, Shaw EG, Pugh SL, Chakravarti A, Gilbert MR, Barger GR, Coons S, Ricci P, Bullard D, Brown PD, Stelzer K, Brachman D, Suh JH, Schultz CJ, Bahary JP, Fisher BJ, Kim H, Murtha AD, Bell EH, Won M, Mehta MP, Curran WJ (April 2016). "Radiation plus Procarbazine, CCNU, and Vincristine in Low-Grade Glioma". N. Engl. J. Med. 374 (14): 1344–55. doi:10.1056/NEJMoa1500925. PMC 5170873. PMID 27050206.
  12. ↑ Shaw EG, Wang M, Coons SW, Brachman DG, Buckner JC, Stelzer KJ, Barger GR, Brown PD, Gilbert MR, Mehta MP (September 2012). "Randomized trial of radiation therapy plus procarbazine, lomustine, and vincristine chemotherapy for supratentorial adult low-grade glioma: initial results of RTOG 9802". J. Clin. Oncol. 30 (25): 3065–70. doi:10.1200/JCO.2011.35.8598. PMC 3732006. PMID 22851558.
  13. ↑ Juratli TA, Lautenschläger T, Geiger KD, Pinzer T, Krause M, Schackert G, Krex D (September 2015). "Radio-chemotherapy improves survival in IDH-mutant, 1p/19q non-codeleted secondary high-grade astrocytoma patients". J. Neurooncol. 124 (2): 197–205. doi:10.1007/s11060-015-1822-1. PMID 26033545.
  14. ↑ Shin JY, Diaz AZ (September 2016). "Anaplastic astrocytoma: prognostic factors and survival in 4807 patients with emphasis on receipt and impact of adjuvant therapy". J. Neurooncol. 129 (3): 557–565. doi:10.1007/s11060-016-2210-1. PMID 27401155.
  15. ↑ Wick W, Hartmann C, Engel C, Stoffels M, Felsberg J, Stockhammer F, Sabel MC, Koeppen S, Ketter R, Meyermann R, Rapp M, Meisner C, Kortmann RD, Pietsch T, Wiestler OD, Ernemann U, Bamberg M, Reifenberger G, von Deimling A, Weller M (December 2009). "NOA-04 randomized phase III trial of sequential radiochemotherapy of anaplastic glioma with procarbazine, lomustine, and vincristine or temozolomide". J. Clin. Oncol. 27 (35): 5874–80. doi:10.1200/JCO.2009.23.6497. PMID 19901110.
  16. ↑ Wick W, Roth P, Hartmann C, Hau P, Nakamura M, Stockhammer F, Sabel MC, Wick A, Koeppen S, Ketter R, Vajkoczy P, Eyupoglu I, Kalff R, Pietsch T, Happold C, Galldiks N, Schmidt-Graf F, Bamberg M, Reifenberger G, Platten M, von Deimling A, Meisner C, Wiestler B, Weller M (November 2016). "Long-term analysis of the NOA-04 randomized phase III trial of sequential radiochemotherapy of anaplastic glioma with PCV or temozolomide". Neuro-oncology. 18 (11): 1529–1537. doi:10.1093/neuonc/now133. PMC 5063521. PMID 27370396.
  17. ↑ Stupp R, Mason WP, van den Bent MJ, Weller M, Fisher B, Taphoorn MJ, Belanger K, Brandes AA, Marosi C, Bogdahn U, Curschmann J, Janzer RC, Ludwin SK, Gorlia T, Allgeier A, Lacombe D, Cairncross JG, Eisenhauer E, Mirimanoff RO (March 2005). "Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma". N. Engl. J. Med. 352 (10): 987–96. doi:10.1056/NEJMoa043330. PMID 15758009.
  18. ↑ Kole AJ, Park HS, Yeboa DN, Rutter CE, Corso CD, Aneja S, Lester-Coll NH, Mancini BR, Knisely JP, Yu JB (August 2016). "Concurrent chemoradiotherapy versus radiotherapy alone for "biopsy-only" glioblastoma multiforme". Cancer. 122 (15): 2364–70. doi:10.1002/cncr.30063. PMID 27172136.
  19. ↑ Lai A, Tran A, Nghiemphu PL, Pope WB, Solis OE, Selch M, Filka E, Yong WH, Mischel PS, Liau LM, Phuphanich S, Black K, Peak S, Green RM, Spier CE, Kolevska T, Polikoff J, Fehrenbacher L, Elashoff R, Cloughesy T (January 2011). "Phase II study of bevacizumab plus temozolomide during and after radiation therapy for patients with newly diagnosed glioblastoma multiforme". J. Clin. Oncol. 29 (2): 142–8. doi:10.1200/JCO.2010.30.2729. PMC 3058273. PMID 21135282.
  20. ↑ Stupp R, Wong ET, Kanner AA, Steinberg D, Engelhard H, Heidecke V, Kirson ED, Taillibert S, Liebermann F, Dbalý V, Ram Z, Villano JL, Rainov N, Weinberg U, Schiff D, Kunschner L, Raizer J, Honnorat J, Sloan A, Malkin M, Landolfi JC, Payer F, Mehdorn M, Weil RJ, Pannullo SC, Westphal M, Smrcka M, Chin L, Kostron H, Hofer S, Bruce J, Cosgrove R, Paleologous N, Palti Y, Gutin PH (September 2012). "NovoTTF-100A versus physician's choice chemotherapy in recurrent glioblastoma: a randomised phase III trial of a novel treatment modality". Eur. J. Cancer. 48 (14): 2192–202. doi:10.1016/j.ejca.2012.04.011. PMID 22608262.
  21. ↑ Westphal M, Hilt DC, Bortey E, Delavault P, Olivares R, Warnke PC, Whittle IR, Jääskeläinen J, Ram Z (April 2003). "A phase 3 trial of local chemotherapy with biodegradable carmustine (BCNU) wafers (Gliadel wafers) in patients with primary malignant glioma". Neuro-oncology. 5 (2): 79–88. doi:10.1093/neuonc/5.2.79. PMC 1920672. PMID 12672279.

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