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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: kidney, liver, lung, breast, and liver. The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma, the mutation results in aberrant angiogenesis. 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.  On CT, findings of angiosarcoma may include: vascular invasion, nodular enhancement (common), and a hypoattenuating mass. 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> However, 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 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: [[kidney]], [[liver]], [[lung]], [[breast]], and [[liver]]. The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma, the mutation results in aberrant angiogenesis. 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.  On CT, findings of angiosarcoma may include: vascular invasion, nodular enhancement (common), and a hypoattenuating mass. 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> However, 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==
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==Pathophysiology==
==Pathophysiology==
*The pathogenesis of angiosarcoma is characterized by a rapid and extensively 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>
*The pathogenesis of angiosarcoma is characterized by a rapid and extensively 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>  
*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: kidney, liver, lung, breast, skin, and liver.
*Common angiosarcoma locations, include: 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>
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:*Red/dark tan lesion
:*Red/dark tan lesion
:*Typically poorly circumscribed
:*Typically poorly circumscribed
*The image below demonstrates gross pathology of angiosarcoma.<ref name="librepathology"> Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>  
*The image below demonstrates gross pathology of angiosarcoma.<ref name="librepathology">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016</ref>  
<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>
</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>  
*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>  
:*Spindle cell lesion.
:*[[Spindle cells|Spindle cell]] lesion.
:*Occasionally an epitheloid lesion
:*Occasionally an epitheloid lesion
:*Very many small capillaries of irregular shape  
:*Very many small capillaries of irregular shape  
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==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 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>
:*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 radioactive compound thorium dioxide
:*Previous exposure to radioactive compound [[thorium dioxide]]


==Differentiating Angiosarcoma from Other Diseases==
==Differentiating Angiosarcoma from Other Diseases==
*Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass, or unhealed skin ulcer (for cutaneous angiosarcoma), 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 unhealed skin ulcer (for cutaneous angiosarcoma), such as:<ref name="wiki">Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed April 22, 2016 </ref>
:*Atypical vascular lesions
:*Atypical vascular lesions
:*Hemangioma
:*[[Hemangioma]]
:*Glomangiosarcoma
:*Glomangiosarcoma
:*Carotid body tumor
:*[[Carotid body tumor]]
:*Malignant fibrous histiocytoma of soft tissue
:*[[Malignant fibrous histiocytoma]] of soft tissue
*Cutaneous angiosarcoma
*Cutaneous angiosarcoma
:*Basocellular skin carcinoma
:*Basocellular skin carcinoma
:*Keratoacanthoma
:*[[Keratoacanthoma]]
:*Nodular melanom
:*[[Nodular melanoma]]
:*Mycosis fungoides
:*[[Mycosis fungoides]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
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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
:*Polyvinyl chloride (PVC)
:*[[Polyvinyl chloride]] (PVC)
:*Radiation exposure
:*[[Radiation exposure]]
:*Thorotrast
:*[[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>
*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 unspecific general symptoms, such as: pain, fatigue, malaise, and nausea.  
*Early clinical features may include unspecific general symptoms, such as: [[pain]], [[fatigue]], [[malaise]], and [[nausea]].  
*If left untreated, the majority of patients with angiosarcoma may progress to rapidly 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 progress to rapidly 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>
:*Pathologic fractures
:*[[Fractures|Pathologic fractures]]
:*Anemia
:*[[Anemia]]
:*Hepatic dysfunction
:*[[Hepatic dysfunction]]
*Prognosis is generally poor and the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%.
*Prognosis is generally poor and the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%.
*Factors related with worse prognosis, include: patient age (> 65), retroperitoneum 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>
*Factors related with worse prognosis, include: patient age (> 65), retroperitoneum 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>
Line 105: Line 105:
*In cutaneous angiosarcoma, remarkable physical examination findings may include:
*In cutaneous angiosarcoma, remarkable physical examination findings may include:
:*Inspection of scalp, chest, or head.  
:*Inspection of scalp, chest, or head.  
:*Bruise or skin ulceration
:*Bruise or [[skin ulceration]]
:*Blushed purplish-red papule
:*Blushed purplish-red papule


Line 131: Line 131:
=== 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>
*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>
*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>
*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>
*Common complications of doxorubicin, include:
*Common complications of doxorubicin, include:
:*Chronic cardiotoxicity
:*Chronic [[cardiotoxicity]]
:*Mucositis
:*[[Mucositis]]
:*Alopecia
:*[[Alopecia]]
:*Nausea
:*[[Nausea]]
:*Vomiting
:*[[Vomiting]]


=== Surgery ===
=== Surgery ===
Line 152: Line 152:
*Follow-up testing for 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>
*Follow-up testing for 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>
:*Periodical imaging/angiography evaluation  
:*Periodical imaging/angiography evaluation  
:*Laboratory testing: complete blood count (eg. anemia)
:*Laboratory testing: complete blood count (eg. [[anemia]])


==References==
==References==

Revision as of 15:47, 22 April 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 Rosai 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: kidney, liver, lung, breast, and liver. The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma, the mutation results in aberrant angiogenesis. 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. On CT, findings of angiosarcoma may include: vascular invasion, nodular enhancement (common), and a hypoattenuating mass. The main adjuvant therapy for angiosarcoma is a doxorubicin-based regimen.[5] However, the response rate for chemotherapy in patients with angiosarcoma is poor.[5]

Historical Perspective

  • Angiosarcoma was first discovered by Rosai 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/uncategorised

Pathophysiology

  • The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular epithelial cells.[5]
  • Angiosarcoma is a locally aggressive tumor with a high rate of lymph node infiltration and metastases.[2]
  • Common angiosarcoma locations, include: kidney, liver, lung, breast, skin, and liver.
  • The most common type of angiosarcoma is cutaneous angiosarcoma.[5]
  • The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma (the result is the aberrant formation of angiogenesis).[5]
  • On gross pathology, characteristic findings of angiosarcoma, may include:[5]
  • Red/dark tan lesion
  • Typically poorly circumscribed
  • The image below demonstrates 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]
  • Spindle cell lesion.
  • Occasionally an epitheloid lesion
  • Very many small capillaries of irregular shape
  • Pleomorphic nuclei
  • May have hobnail morphology
  • Usually "red" at low power (key feature)
  • Mitoses
  • Cytoplasmic vacuoles
  • Cells trying to form lumina

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 unhealed skin ulcer (for cutaneous angiosarcoma), such as:[6]
  • Cutaneous angiosarcoma

Epidemiology and Demographics

  • Angiosarcoma is uncommon.
  • 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.

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 unspecific general symptoms, such as: pain, fatigue, malaise, and nausea.
  • If left untreated, the majority of patients with angiosarcoma may progress to rapidly develop metastases.[4]
  • Common complications of angiosarcoma, include:[5]
  • Prognosis is generally poor and the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%.
  • Factors related with worse prognosis, include: patient age (> 65), retroperitoneum 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 usually appear cachectic, or normal.
  • In cutaneous angiosarcoma, remarkable physical examination findings may include:
  • Inspection of scalp, chest, or head.
  • Bruise or skin ulceration
  • Blushed purplish-red papule

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.
  • On CT, findings of angiosarcoma may include:[4]
  • Vascular invasion
  • Nodular enhancement (common)
  • Hypoattenuating mass
  • Multicentric

On MRI, findings of angiosarcoma may include:

  • T1/T2: heterogeneous areas of high signal reflecting mixed tumour and haemorrhage
  • T1 C+ (Gd): heterogeneous enhancement

Gallery

  • CT Pulmonary angiosarcoma Courtesy of Radiopedia
    CT Pulmonary angiosarcoma
    Courtesy of Radiopedia

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, combination of radiotherapy and immunotherapy with recombinant interleukin-2 is the treatment of choice.[7]
  • The response rate for chemotherapy in patients with angiosarcoma is poor.[5]
  • Common complications of doxorubicin, include:

Surgery

  • Surgical resection in combination with radiation therapy is the treatment of choice for small angiosarcoma.[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 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, include:[5]
  • Periodical imaging/angiography evaluation
  • Laboratory testing: complete blood count (eg. anemia)

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 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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