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Synonyms and Keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma
Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls. Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976. 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. 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. The response rate for chemotherapy in patients with angiosarcoma is poor.
Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.
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
The pathogenesis of angiosarcoma is characterized by a rapid and extensive infiltrating overgrowth of vascular epithelial cells. Angiosarcoma is a locally aggressive tumor with a high rate of lymph node infiltration and metastases.
On gross pathology, characteristic findings of angiosarcoma may include:
- Red/dark tan lesion
- Typically poorly circumscribed
Examples of Gross Pathology
Gross pathology: angiosarcoma
Courtesy of Libre Pathology 
On microscopic histopathological analysis, characteristic findings of angiosarcoma may include:
- 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
- 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:
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
- In 2004, the age-adjusted incidence of angiosarcoma was 3.1 per 100,000 population per year.
- Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.
- Males are more commonly affected with angiosarcoma than females.
- The male to female ratio is 2:1.
- There is no racial predilection for angiosarcoma.
Common risk factors in the development of angiosarcoma include:
- 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. 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.
Common complications of angiosarcoma include:
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.
Angiosarcoma is usually asymptomatic and found incidentally. Symptoms of angiosarcoma are generally non-specific.
Patients with angiosarcoma may appear cachectic or normal. In cutaneous angiosarcoma, physical examination findings may include:
There are no specific laboratory findings associated with angiosarcoma.
The imaging modality of choice for angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is enhanced CT scan. For other types angiosarcoma, the imaging modality of choice is MRI.
On CT, findings of angiosarcoma may include:
- Vascular invasion
- Nodular enhancement (common)
- Hypoattenuating mass
- Multicentric lesions
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
The mainstay of treatment for angiosarcoma is complete surgical resection with wide margins for local and locoregional disease in combination with preoperative or postoperative radiotherapy. The role of adjuvant chemotherapy, is unclear.Adjuvant chemotherapy and/or radiotheray provide less mutilating surgery, and for patients with unresectable tumors or those who refuse surgery is an option.
- 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. The response rate to chemotherapy in patients with angiosarcoma is poor.
- Surgical treatment for patients with cutaneous angiosarcoma is surgical resection with wide margins. Surgery is not recommended on patients with large sized angiosarcomas. The recurrence rate of angiosarcoma after surgery is 80%.
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:
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