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==Overview==
==Overview==


'''Angiosarcoma''' is a rare [[malignant]] vascular [[neoplasm]] of [[endothelial]]-type cells that line vessel walls.<ref name="Robbins">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.</ref><ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref> Angiosarcoma was first discovered by Rosai 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> 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]] /[[Phospholipase C|PLCG1]] [[genes]] is 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.<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. 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.<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 for [[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>
'''Angiosarcoma''' is a rare [[malignant]] vascular [[neoplasm]] of [[endothelial]]-type cells that line vessel walls.<ref name="Robbins">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.</ref><ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</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> 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]]/[[Phospholipase C|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.<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. 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.<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 for [[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>


==Historical Perspective==
==Historical Perspective==
*Angiosarcoma was first discovered by Rosai 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==
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*Common angiosarcoma locations include the [[kidney]], [[liver]], [[lung]], [[breast]], [[skin]], and [[liver]].
*Common angiosarcoma locations include the [[kidney]], [[liver]], [[lung]], [[breast]], [[skin]], and [[liver]].
*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 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>
*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
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==Differentiating Angiosarcoma from Other Diseases==
==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:<ref name="wiki">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed April 22, 2016 </ref>
Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass or non-healing cutaneous ulcerations such as:<ref name="wiki">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed April 22, 2016 </ref>
===Differentials for Cutaneous Angiosarcoma===
===Differentials for Cutaneous Angiosarcoma===
Cutaneous angiosarcoma must be differentiated from other diseases with non-healing cutaneous ulcerations such as:
Cutaneous angiosarcoma must be differentiated from other diseases with non-healing cutaneous ulcerations such as:
:*Basocellular skin carcinoma
:*[[Basal cell carcinoma]]
:*[[Keratoacanthoma]]
:*[[Keratoacanthoma]]
:*[[Nodular melanoma]]
:*[[Nodular melanoma]]
:*[[Mycosis fungoides]]
:*[[Mycosis fungoides]]
===Differentials for Non-cutaneous Angiosarcoma===
===Differentials for Non-cutaneous Angiosarcoma===
Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass such as:
Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass such as:
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:*[[Carotid body tumor]]
:*[[Carotid body tumor]]
:*[[Malignant fibrous histiocytoma]] of soft tissue
:*[[Malignant fibrous histiocytoma]] of soft tissue


==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Incidence===
In 2004, the age-adjusted incidence of angiosarcoma was 3.1 per 100,000 population per year.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
In 2004, the age-adjusted incidence of angiosarcoma was 3.1 per 100,000 population per year.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
===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>
*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===
*Males are more commonly affected with angiosarcoma than females.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
*Males are more commonly affected with angiosarcoma than females.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
*The male to female ratio is 2:1.  
*The male to female ratio is 2:1.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
 
===Race===
===Race===
*There is no racial predilection for angiosarcoma.
*There is no racial predilection for angiosarcoma.
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*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]]
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*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>
*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 nonnspecific symptoms, such as [[pain]], [[fatigue]], [[malaise]], and [[nausea]].  
*Early clinical features may include nonnspecific 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>
*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]]
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* 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 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 other types angiosarcoma, the imaging modality of choice is MRI.
====CT findings====
====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>
:*Vascular invasion  
*Vascular invasion  
:*Nodular enhancement (common)
*Nodular enhancement (common)
:*Hypoattenuating mass
*Hypoattenuating mass
:*Multicentric lesions
*Multicentric lesions
====MRI findings====
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


====Examples of Imaging Findings====
<gallery>
<gallery>
Image: Pulmonary-angiosarcoma.jpg | CT Pulmonary angiosarcoma <br> <SMALL> Courtesy of Radiopedia</SMALL>
Image: Pulmonary-angiosarcoma.jpg | CT Pulmonary angiosarcoma <br> <SMALL> Courtesy of Radiopedia</SMALL>
</gallery>
</gallery>
====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 ==
== 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>
*For patients with pulmonary angiosarcoma, combination of radiotherapy and 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>
:*Common complications of [[doxorubicin]] include:
::*[[Cardiotoxicity]]
::*[[Mucositis]]
::*[[Alopecia]]
::*[[Nausea]]
::*[[Vomiting]]
 
*For patients with pulmonary angiosarcoma, a combination of [[radiotherapy]] and [[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>
*Common complications of [[doxorubicin]] include:
:*[[Cardiotoxicity]]
:*[[Mucositis]]
:*[[Alopecia]]
:*[[Nausea]]
:*[[Vomiting]]


=== Surgery ===
=== Surgery ===
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*There are no primary preventive measures available for angiosarcoma.
*There are no primary preventive measures available for angiosarcoma.
*Once diagnosed and successfully treated, patients with angiosarcoma are followed-up every 3, 6, or 12 months depending on the stage at diagnosis.  
*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 includes:<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>
*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>
:*Periodic imaging/[[angiography]] evaluation  
:*Periodic imaging/[[angiography]] evaluation  
:*Laboratory testing: [[complete blood count]] (eg. [[anemia]])
:*Laboratory testing: [[complete blood count]] (e.g., [[anemia]])


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
   
   
[[Category: Oncology]]
[[Category:Oncology]]
[[Category: FinalQCRequired]]
[[Category:FinalQCRequired]]

Revision as of 13:30, 5 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
  • Liver angiosarcomas
  • Lung angiosarcomas
  • Spleen angiosarcomas
  • Others/uncategorized

Pathophysiology

  • Red/dark tan lesion
  • Typically poorly circumscribed
  • The following is an example of the gross pathology of angiosarcoma:[2]
  • Gross pathology: angiosarcoma Courtesy of Libre Pathology

    Gross pathology: angiosarcoma
    Courtesy of Libre Pathology

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

Causes

  • Common causes of angiosarcoma may 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]
  • Angiosarcoma is more commonly observed among middle aged adults and elder patients.[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

  • The majority of patients with angiosarcoma remain asymptomatic for years.[5]
  • Early clinical features may include nonnspecific symptoms, such as pain, fatigue, malaise, and nausea.
  • If left untreated, the majority of patients with angiosarcoma may rapidly progress to develop metastases.[4]
  • Common complications of angiosarcoma include:[5]
  • 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.
  • There are no remarkable symptoms for angiosarcoma.

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

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

  • There are no primary preventive measures available for angiosarcoma.
  • 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 4.9 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 5.15 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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