Angiosarcoma

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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, and breast. The PTPRB/PLCG1 genes are associated with the development of angiosarcoma; mutation of these genes result 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

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

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. PMC 3649120Freely accessible. PMID 24946325. doi:10.1093/jscr/2010.5.7. 
  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. PMID 20537949. doi:10.1016/S1470-2045(10)70023-1.  Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid20537949" defined multiple times with different content
  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. PMID 15249484. doi:10.1378/chest.126.1.317. 

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