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]; Tarek Nafee, M.D. [3]
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
- 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]
- Common angiosarcoma locations include the kidney, liver, lung, breast, skin, and liver.
- The most common type of angiosarcoma is cutaneous angiosarcoma.[5]
- The PTPRB/PLCG1 genes are associated with the development of angiosarcoma by triggering aberrant angiogenesis.[5]
- On gross pathology, characteristic findings of angiosarcoma may include:[5]
- 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
- On microscopic histopathological analysis, characteristic findings of angiosarcoma may include:[2]
- Spindle cell lesion
- Epitheloid lesion
- Numerous irrergular capillaries
- Appears red on low power
- Pleomorphic nuclei
- Hobnail morphology
- Numerous mitotic bodies
- Cytoplasmic vacuoles
- Luminal arrangement of cells
Causes
- Exposure to vinyl chloride monomer (VCM) for prolonged periods
- Exposure to polyvinyl chloride (PVC) polymerisation plants
- Exposure to arsenic-containing insecticides
- Previous exposure to thorium dioxide irradiation
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:
- Atypical vascular lesions
- Hemangioma
- Glomangiosarcoma
- Carotid body tumor
- Malignant fibrous histiocytoma of soft tissue
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]
- Chronic lymphedema
- Chronic exposure to polyvinyl chloride (PVC)
- Radiation exposure
- Exposure to Thorotrast
Natural History, Complications and Prognosis
- 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]
- 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:
- Bruise or skin ulceration
- Blushed purple-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.
CT
On CT, findings of angiosarcoma may include:[4]
- Vascular invasion
- Nodular enhancement (common)
- Hypoattenuating mass
- Multicentric lesions
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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]
- Common complications of doxorubicin include:
- 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
- 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]
- Periodic imaging/angiography evaluation
- Laboratory testing: complete blood count (e.g., anemia)
References
- ↑ 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.
- ↑ 2.0 2.1 2.2 2.3 2.4 Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed on April 22, 2016
- ↑ 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.
- ↑ 4.0 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
- ↑ 5.00 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.
- ↑ Angiosarcoma. Wikipedia. https://en.wikipedia.org/wiki/Angiosarcoma Accessed April 22, 2016
- ↑ 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.