Angiosarcoma: Difference between revisions

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{{SI}}                                                                 
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{{CMG}}; {{AE}} {{MV}}; {{TarekNafee}}
{{CMG}}; {{AE}} {{MV}}
   
   
{{SK}}  Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma  
{{SK}}  Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma  
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==Historical Perspective==
==Historical Perspective==
*Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.<ref name="pmid24946325">{{cite journal |vauthors=Barber W, Scriven P, Turner D, Hughes D, Wyld D |title=Epithelioid angiosarcoma: Use of angiographic embolisation and radiotherapy to control recurrent haemorrhage |journal=J Surg Case Rep |volume=2010 |issue=5 |pages=7 |year=2010 |pmid=24946325 |pmc=3649120 |doi=10.1093/jscr/2010.5.7 |url=}}</ref>
Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.<ref name="pmid24946325">{{cite journal |vauthors=Barber W, Scriven P, Turner D, Hughes D, Wyld D |title=Epithelioid angiosarcoma: Use of angiographic embolisation and radiotherapy to control recurrent haemorrhage |journal=J Surg Case Rep |volume=2010 |issue=5 |pages=7 |year=2010 |pmid=24946325 |pmc=3649120 |doi=10.1093/jscr/2010.5.7 |url=}}</ref>


==Classification==
==Classification==
*Angiosarcoma may be classified according to anatomical location into the following categories:  
Angiosarcoma may be classified according to anatomical location into the following categories:  
 
*Head and neck angiosarcomas
:*Head and neck angiosarcomas
*Skin angiosarcomas (Most common)
:*Skin angiosarcomas
*Liver angiosarcomas
:*Liver angiosarcomas
*Lung angiosarcomas
:*Lung angiosarcomas
*Spleen angiosarcomas  
:*Spleen angiosarcomas  
*Others/uncategorized
:*Others/uncategorized


==Pathophysiology==
==Pathophysiology==
*The pathogenesis of angiosarcoma is characterized by a rapid and extensive infiltrating overgrowth of vascular [[epithelial cells]].<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
===Pathogenesis===
*Angiosarcoma is a locally aggressive [[tumor]] with a high rate of [[lymph node]] infiltration and [[metastases]].<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>  
The pathogenesis of angiosarcoma is characterized by a rapid and extensive infiltrating overgrowth of vascular [[epithelial cells]].<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref> Angiosarcoma is a locally aggressive [[tumor]] with a high rate of [[lymph node]] infiltration and [[metastases]].<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>  
*Common angiosarcoma locations include the [[kidney]], [[liver]], [[lung]], [[breast]], [[skin]], and [[liver]].
===Genetics===
*The most common type of angiosarcoma is [[cutaneous]] angiosarcoma.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
The [[PTPRB]]/[[Phospholipase C|PLCG1]] [[genes]] are associated with the development of angiosarcoma by triggering aberrant [[angiogenesis]].<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*The [[PTPRB]]/[[Phospholipase C|PLCG1]] [[genes]] are associated with the development of angiosarcoma by triggering aberrant [[angiogenesis]].<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
===Gross Pathology===
*On gross pathology, characteristic findings of angiosarcoma may include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
On gross pathology, characteristic findings of angiosarcoma may include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
:*Red/dark tan lesion
*Red/dark tan lesion
:*Typically poorly circumscribed
*Typically poorly circumscribed
*The following is an example of the gross pathology of angiosarcoma:<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>
====Examples of Gross Pathology====  
<gallery>
<gallery>
Image: Angiosarcoma gross pathology.jpg| Gross pathology: angiosarcoma<br> <SMALL> Courtesy of Libre Pathology </SMALL>
Image: Angiosarcoma gross pathology.jpg| Gross pathology: angiosarcoma<br> <SMALL> Courtesy of Libre Pathology </SMALL><ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>
</gallery>
</gallery>
*On microscopic histopathological analysis, characteristic findings of angiosarcoma may include:<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>  
===Microscopic Pathology===
:*[[Spindle cells|Spindle cell]] lesion
On microscopic histopathological analysis, characteristic findings of angiosarcoma may include:<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>  
:*Epitheloid lesion
*[[Spindle cells|Spindle cell]] lesion
:*Numerous irrergular [[capillaries]]
*Epitheloid lesion
:*Appears red on low power
*Numerous irrergular [[capillaries]]
:*[[Pleomorphic]] nuclei  
*Appears red on low power
:*Hobnail morphology
*[[Pleomorphic]] nuclei  
:*Numerous [[mitotic]] bodies
*Hobnail morphology
:*[[Cytoplasmic]] [[vacuoles]]
*Numerous [[mitotic]] bodies
:*[[Lumen (anatomy)|Luminal]] arrangement of cells
*[[Cytoplasmic]] [[vacuoles]]
*[[Lumen (anatomy)|Luminal]] arrangement of cells


==Causes==
==Causes==
*Common causes of angiosarcoma may include:<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref><ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
Common causes of angiosarcoma include:<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref><ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
:*Exposure to [[vinyl chloride]] monomer (VCM) for prolonged periods  
*Exposure to [[vinyl chloride]] monomer (VCM) for prolonged periods  
:*Exposure to [[polyvinyl chloride]] (PVC) polymerisation plants
*Exposure to [[polyvinyl chloride]] (PVC) polymerisation plants
:*Exposure to [[arsenic]]-containing [[insecticides]]  
*Exposure to [[arsenic]]-containing [[insecticides]]  
:*Previous exposure to [[thorium dioxide]] [[irradiation]]
*Previous exposure to [[thorium dioxide]] [[irradiation]]


==Differentiating Angiosarcoma from Other Diseases==
==Differentiating Angiosarcoma from Other Diseases==
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===Age===  
===Age===  
*Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
*Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
*Angiosarcoma is more commonly observed among middle aged adults and elder patients.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>


===Gender===
===Gender===
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===Race===
===Race===
*There is no racial predilection for angiosarcoma.
There is no racial predilection for angiosarcoma.


==Risk Factors==
==Risk Factors==
*Common risk factors in the development of angiosarcoma include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
Common risk factors in the development of angiosarcoma include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
:*Chronic lymphedema
*Chronic lymphedema
:*Chronic exposure to [[polyvinyl chloride]] (PVC)
*Chronic exposure to [[polyvinyl chloride]] (PVC)
:*[[Radiation exposure]]
*[[Radiation exposure]]
:*Exposure to [[Thorotrast]]
*Exposure to [[Thorotrast]]


== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with angiosarcoma remain asymptomatic for years.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
===Natural History===
*Early clinical features may include nonspecific symptoms, such as [[pain]], [[fatigue]], [[malaise]], and [[nausea]].  
The majority of patients with angiosarcoma remain asymptomatic for years.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref> Early clinical features may include nonspecific symptoms, such as [[pain]], [[fatigue]], [[malaise]], and [[nausea]]. If left untreated, the majority of patients with angiosarcoma may rapidly progress to develop [[metastases]].<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
*If left untreated, the majority of patients with angiosarcoma may rapidly progress to develop [[metastases]].<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
===Complications===
*Common complications of angiosarcoma include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
Common complications of angiosarcoma include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
:*[[Fractures|Pathologic fractures]]
*[[Fractures|Pathologic fractures]]
:*[[Anemia]]
*[[Anemia]]
:*[[Hepatic dysfunction]]
*[[Hepatic dysfunction]]
*Prognosis is generally poor; the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%.
===Prognosis===
*Poor prognostic factors include patient age (> 65 years), [[retroperitoneal]] location, and large [[tumor]] size.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
Prognosis is generally poor; the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%. *Poor prognostic factors include patient age (> 65 years), [[retroperitoneal]] location, and large [[tumor]] size.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>


== Diagnosis ==
== Diagnosis ==
=== Symptoms ===
=== Symptoms ===
*Angiosarcoma is usually asymptomatic and found incidentally.  
Angiosarcoma is usually asymptomatic and found incidentally. Symptoms of angiosarcoma are generally non-specific.
*There are no remarkable symptoms for angiosarcoma.  
 
=== Physical Examination ===
=== Physical Examination ===
*Patients with angiosarcoma may appear [[Cachexia|cachectic]] or normal.
Patients with angiosarcoma may appear [[Cachexia|cachectic]] or normal. In cutaneous angiosarcoma, physical examination findings may include:
*In cutaneous angiosarcoma, physical examination findings may include:
*Bruise or [[skin ulceration]]
:*Bruise or [[skin ulceration]]
*Blushed purple-red [[papule]]
:*Blushed purple-red [[papule]]
 
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with angiosarcoma.
There are no specific laboratory findings associated with angiosarcoma.
 
===Imaging Findings===
===Imaging Findings===
*The imaging modality of choice for angiosarcoma will depend on the location.  
The imaging modality of choice for angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is enhanced CT scan.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref> For other types angiosarcoma, the imaging modality of choice is MRI.
* For pulmonary angiosarcoma, the imaging modality of choice is enhanced CT scan.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
* For other types angiosarcoma, the imaging modality of choice is MRI.
====CT====
====CT====
On CT, findings of angiosarcoma may include:<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
On CT, findings of angiosarcoma may include:<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
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====MRI====
====MRI====
On MRI, findings of angiosarcoma may include:
On MRI, findings of angiosarcoma may include:
:*T1/T2: heterogeneous areas of hyperintensity suggestive of a mixed tumour and [[hemorrhage]]
*T1/T2: heterogeneous areas of hyperintensity suggestive of a mixed tumour and [[hemorrhage]]
:*T1 C+ (Gd): heterogeneous enhancement
*T1 C+ (Gd): heterogeneous enhancement


== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*The main [[adjuvant therapy]] for angiosarcoma is a [[doxorubicin]]-based regimen.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
The main [[adjuvant therapy]] for angiosarcoma is a [[doxorubicin]]-based regimen.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref> For angiosarcoma, [[doxorubicin]] monotherapy is indicated as first-line therapy.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*For angiosarcoma, [[doxorubicin]] monotherapy is indicated as first-line therapy.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*Common complications of [[doxorubicin]] include:
:*Common complications of [[doxorubicin]] include:
:*[[Cardiotoxicity]]
::*[[Cardiotoxicity]]
:*[[Mucositis]]
::*[[Mucositis]]
:*[[Alopecia]]
::*[[Alopecia]]
:*[[Nausea]]
::*[[Nausea]]
:*[[Vomiting]]
::*[[Vomiting]]


*For patients with pulmonary angiosarcoma, a combination of [[radiotherapy]] and [[cancer immunotherapy|immunotherapy]] with recombinant [[interleukin-2]] is the treatment of choice.<ref name="pmid15249484">{{cite journal |vauthors=Duck L, Baurain JF, Machiels JP |title=Treatment of a primary pulmonary angiosarcoma |journal=Chest |volume=126 |issue=1 |pages=317–8; author reply 318 |year=2004 |pmid=15249484 |doi=10.1378/chest.126.1.317 |url=}}</ref>
For patients with pulmonary angiosarcoma, a combination of [[radiotherapy]] and [[cancer immunotherapy|immunotherapy]] with recombinant [[interleukin-2]] is the treatment of choice.<ref name="pmid15249484">{{cite journal |vauthors=Duck L, Baurain JF, Machiels JP |title=Treatment of a primary pulmonary angiosarcoma |journal=Chest |volume=126 |issue=1 |pages=317–8; author reply 318 |year=2004 |pmid=15249484 |doi=10.1378/chest.126.1.317 |url=}}</ref> The response rate to [[chemotherapy]] in patients with angiosarcoma is poor.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*The response rate to [[chemotherapy]] in patients with angiosarcoma is poor.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>


=== Surgery ===
=== Surgery ===
*Surgical resection in combination with [[radiation therapy]] is the treatment of choice for small angiosarcomas.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
Surgical resection in combination with [[radiation therapy]] is the treatment of choice for small angiosarcomas.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*Surgical treatment for patients with cutaneous angiosarcoma is surgical resection with wide margins.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*Surgical treatment for patients with cutaneous angiosarcoma is surgical resection with wide margins.<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref> Surgery is not recommended on patients with large sized angiosarcomas. The recurrence rate of angiosarcoma after surgery is 80%.
*Surgery is not recommended on patients with large sized angiosarcomas.  
*The recurrence rate of angiosarcoma after surgery is 80%.


=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for angiosarcoma.
====Primary Prevention====
*Once diagnosed and successfully treated, patients with angiosarcoma are followed-up every 3, 6, or 12 months depending on the stage at diagnosis.  
There are no primary preventive measures available for angiosarcoma.
*Follow-up testing for angiosarcoma may include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
====Secondary Prevention====
:*Periodic imaging/[[angiography]] evaluation  
Once diagnosed and successfully treated, patients with angiosarcoma are followed-up every 3, 6, or 12 months depending on the stage at diagnosis. Follow-up testing for angiosarcoma may include:<ref name="pmid20537949">{{cite journal |vauthors=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol. |volume=11 |issue=10 |pages=983–91 |year=2010 |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
:*Laboratory testing: [[complete blood count]] (e.g., [[anemia]])
*Periodic imaging/[[angiography]] evaluation  
*Laboratory testing: [[complete blood count]] (e.g., [[anemia]])


==References==
==References==
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[[Category:Oncology]]
[[Category:Oncology]]
[[Category:FinalQCRequired]]
[[Category:FinalQCRequired]]
{{WH}}{{WS}}

Revision as of 19:00, 24 August 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma

Overview

Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls.[1][2] Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.[3] The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular epithelial cells. Common angiosarcoma locations include the kidney, liver, lung, breast, and liver. The PTPRB/PLCG1 genes are associated with the development of angiosarcoma; the mutation results in aberrant angiogenesis. The imaging modality of choice for diagnosing angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is enhanced CT scan.[4] For other types angiosarcoma, the imaging modality of choice is MRI. On CT scan, findings suggestive of angiosarcoma may include vascular invasion, nodular enhancement (common), and a hypoattenuating mass. The mainstay adjuvant therapy for angiosarcoma is a doxorubicin-based regimen.[5] The response rate for chemotherapy in patients with angiosarcoma is poor.[5]

Historical Perspective

Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.[3]

Classification

Angiosarcoma may be classified according to anatomical location into the following categories:

  • Head and neck angiosarcomas
  • Skin angiosarcomas (Most common)
  • Liver angiosarcomas
  • Lung angiosarcomas
  • Spleen angiosarcomas
  • Others/uncategorized

Pathophysiology

Pathogenesis

The pathogenesis of angiosarcoma is characterized by a rapid and extensive infiltrating overgrowth of vascular epithelial cells.[5] Angiosarcoma is a locally aggressive tumor with a high rate of lymph node infiltration and metastases.[2]

Genetics

The PTPRB/PLCG1 genes are associated with the development of angiosarcoma by triggering aberrant angiogenesis.[5]

Gross Pathology

On gross pathology, characteristic findings of angiosarcoma may include:[5]

  • Red/dark tan lesion
  • Typically poorly circumscribed

Examples of Gross Pathology

  • Gross pathology: angiosarcoma Courtesy of Libre Pathology [2]

    Gross pathology: angiosarcoma
    Courtesy of Libre Pathology [2]

Microscopic Pathology

On microscopic histopathological analysis, characteristic findings of angiosarcoma may include:[2]

Causes

Common causes of angiosarcoma include:[2][5]

Differentiating Angiosarcoma from Other Diseases

Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass or non-healing cutaneous ulcerations such as:[6]

Differentials for Cutaneous Angiosarcoma

Cutaneous angiosarcoma must be differentiated from other diseases with non-healing cutaneous ulcerations such as:

Differentials for Non-cutaneous Angiosarcoma

Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass such as:

Epidemiology and Demographics

Incidence

In 2004, the age-adjusted incidence of angiosarcoma was 3.1 per 100,000 population per year.[4]

Age

  • Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.[4]

Gender

  • Males are more commonly affected with angiosarcoma than females.[4]
  • The male to female ratio is 2:1.[4]

Race

There is no racial predilection for angiosarcoma.

Risk Factors

Common risk factors in the development of angiosarcoma include:[5]

Natural History, Complications and Prognosis

Natural History

The majority of patients with angiosarcoma remain asymptomatic for years.[5] Early clinical features may include nonspecific symptoms, such as pain, fatigue, malaise, and nausea. If left untreated, the majority of patients with angiosarcoma may rapidly progress to develop metastases.[4]

Complications

Common complications of angiosarcoma include:[5]

Prognosis

Prognosis is generally poor; the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%. *Poor prognostic factors include patient age (> 65 years), retroperitoneal location, and large tumor size.[4]

Diagnosis

Symptoms

Angiosarcoma is usually asymptomatic and found incidentally. Symptoms of angiosarcoma are generally non-specific.

Physical Examination

Patients with angiosarcoma may appear cachectic or normal. In cutaneous angiosarcoma, physical examination findings may include:

Laboratory Findings

There are no specific laboratory findings associated with angiosarcoma.

Imaging Findings

The imaging modality of choice for angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is enhanced CT scan.[4] For other types angiosarcoma, the imaging modality of choice is MRI.

CT

On CT, findings of angiosarcoma may include:[4]

  • Vascular invasion
  • Nodular enhancement (common)
  • Hypoattenuating mass
  • Multicentric lesions
  • CT Pulmonary angiosarcoma Courtesy of Radiopedia

    CT Pulmonary angiosarcoma
    Courtesy of Radiopedia

MRI

On MRI, findings of angiosarcoma may include:

  • T1/T2: heterogeneous areas of hyperintensity suggestive of a mixed tumour and hemorrhage
  • T1 C+ (Gd): heterogeneous enhancement

Treatment

Medical Therapy

The main adjuvant therapy for angiosarcoma is a doxorubicin-based regimen.[5] For angiosarcoma, doxorubicin monotherapy is indicated as first-line therapy.[5]

For patients with pulmonary angiosarcoma, a combination of radiotherapy and immunotherapy with recombinant interleukin-2 is the treatment of choice.[7] The response rate to chemotherapy in patients with angiosarcoma is poor.[5]

Surgery

Surgical resection in combination with radiation therapy is the treatment of choice for small angiosarcomas.[5]

  • Surgical treatment for patients with cutaneous angiosarcoma is surgical resection with wide margins.[5] Surgery is not recommended on patients with large sized angiosarcomas. The recurrence rate of angiosarcoma after surgery is 80%.

Prevention

Primary Prevention

There are no primary preventive measures available for angiosarcoma.

Secondary Prevention

Once diagnosed and successfully treated, patients with angiosarcoma are followed-up every 3, 6, or 12 months depending on the stage at diagnosis. Follow-up testing for angiosarcoma may include:[5]

References

  1. ↑ Perkins, [edited by] Vinay Kumar, Abul K. Abbas, Jon C. Aster ; artist, James A. (2013). Robbins basic pathology (9th ed. ed.). Philadelphia, PA: Elsevier/Saunders. ISBN 9781437717815.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016
  3. ↑ 3.0 3.1 Barber W, Scriven P, Turner D, Hughes D, Wyld D (2010). "Epithelioid angiosarcoma: Use of angiographic embolisation and radiotherapy to control recurrent haemorrhage". J Surg Case Rep. 2010 (5): 7. doi:10.1093/jscr/2010.5.7. PMC 3649120. PMID 24946325.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52
  5. ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 5.11 5.12 5.13 5.14 Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ (2010). "Angiosarcoma". Lancet Oncol. 11 (10): 983–91. doi:10.1016/S1470-2045(10)70023-1. PMID 20537949.
  6. ↑ Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed April 22, 2016
  7. ↑ Duck L, Baurain JF, Machiels JP (2004). "Treatment of a primary pulmonary angiosarcoma". Chest. 126 (1): 317–8, author reply 318. doi:10.1378/chest.126.1.317. PMID 15249484.

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