Overview
What is the pathophysiology of an angiosarcoma (AS)?
What is the prevalence of angiosarcoma (AS) in the US?
What is the global prevalence of angiosarcoma (AS)?
What are the mortality rates for angiosarcoma (AS)?
Which patient groups are at highest risk for angiosarcoma (AS)?
Presentation
Which is the focus of the clinical history for angiosarcoma (AS)?
What are the risk factors for angiosarcoma (AS)?
What are the signs and symptoms of angiosarcoma (AS)?
Which physical findings are characteristic of angiosarcoma (AS)?
Which physical findings are characteristic of soft tissue angiosarcoma (AS)?
Which physical findings are characteristic of angiosarcoma (AS) of bone?
What are the variants of cutaneous angiosarcoma (AS)?
Which physical findings are characteristic of angiosarcoma (AS) of the scalp and face?
Which clinical history findings are characteristic of lymphedema-associated angiosarcoma (LAS)?
Which clinical history findings are characteristic of radiation-induced angiosarcoma (AS)?
Which clinical history findings are characteristic of epithelioid angiosarcoma (AS)?
What causes angiosarcoma (AS)?
What is the role of radical mastectomy in the etiology of angiosarcoma (AS)?
What is the role of radiotherapy in the etiology of angiosarcoma (AS)?
Which foreign material in the body may cause angiosarcoma (AS)?
What is the role of environmental carcinogens in the etiology of angiosarcoma (AS)?
Which genetic disorders are associated with angiosarcoma?
DDX
Which conditions should be considered in the differential diagnoses of angiosarcoma (AS)?
What are the differential diagnoses for Angiosarcoma?
Workup
What is the role of lab testing in the diagnosis of angiosarcoma (AS)?
What is the role of imaging studies in the diagnosis of soft tissue angiosarcoma (AS)?
What is the role of imaging studies in the diagnosis of bone angiosarcoma (AS)?
What is the role of imaging studies in the diagnosis of cutaneous angiosarcoma (AS)?
What is the role of biopsy in the diagnosis of angiosarcoma (AS)?
Which histologic findings are characteristic of angiosarcoma (AS)?
What is the role of immunohistochemical staining in the diagnosis of angiosarcoma (AS)?
How is angiosarcoma (AS) staged?
What are the stages of bone and soft tissue angiosarcoma (AS)?
Treatment
How is soft-tissue angiosarcoma (AS) treated?
What is the efficacy of chemotherapy for the treatment of soft-tissue angiosarcoma (AS)?
What is the role of radiotherapy in the treatment of soft-tissue angiosarcoma (AS)?
How is bone angiosarcoma (AS) treated?
How is cutaneous angiosarcoma (AS) treated?
What is the role of surgery in the treatment of soft-tissue angiosarcoma (AS)?
What is the role of surgery in the treatment of angiosarcoma (AS) of bone?
What is the role of surgery in the treatment of cutaneous angiosarcoma (AS)?
Medications
What is the goal of drug treatment for angiosarcoma (AS)?
Which medications in the drug class Antineoplastic agents are used in the treatment of Angiosarcoma?
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This is a classic example of angiosarcoma associated with chronic lymphedema after lymphadenectomy and radiotherapy. The patient is a 33-year-old woman who presented with a recurrent grade 2 angiosarcoma of the soft tissue after an attempt at wide excision and skin graft. Two weeks following this procedure, she developed multiple subcutaneous erythematous nodules involving the back and overlying the right scapula region, the supraclavicular fossa, the right breast, and arm. The window shows a microphotograph of the tissue obtained from the biopsy (hematoxylin and eosin stain, original magnification X160). Histologic preparation reveals neoplastic endothelial cells showing a solid pattern with occasional mitotic figures and sporadic protruding growth into the vascular lumens.
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This is a gross specimen from a proximal humerus bone angiosarcoma. These tumors generally are red and hemorrhagic. Although the tumor has not extended into the adjacent soft tissues, cortical erosion is evident. The patient was treated with wide excision and reconstruction with a humeral spacer. The patient's next oncologic recheck showed multiple lesions, including a large destructive lesion in the right ilium extending to the sacroiliac joint and the right sacral ala. Also noted was an upper thoracic vertebral lesion, multiple indeterminate pulmonary nodules, extensive hepatic metastases most marked in the left lobe, and an indeterminate left adnexal mass.
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This radiograph is from a 27-year-old woman who had preexisting mild pain in her left clavicle for a couple of weeks. She woke up one morning with severe pain. Radiographs showed a pathological fracture through a lytic lesion. The permanent section of the needle biopsy is shown in the window and was read as a grade 1 angiosarcoma. The cell morphology varied from epithelioid to spindle-shaped with indistinct borders. Most nuclei were large and vesicular, containing irregular large eosinophilic nucleoli and frequent mitoses. Physicians also found anastomosing vascular channels lined by pleomorphic malignant cells.
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CT scan, MRI, and bone scan from a 27-year-old woman with grade 1 angiosarcoma who presented with a pathological fracture through a lytic lesion on her left clavicle. Note the expansile lytic lesion in the left clavicle accompanied by a soft tissue mass visible on the MRI. No other lesions are identifiable. Regional lymph nodes are visible by MRI, and they do not appear involved. Lungs are clear. She was treated with wide resection of the clavicle, leaving both bony ends. No further reconstruction was attempted. Eighteen months after surgery, she is free of disease and has a full range of motion in the left shoulder. The residual aesthetic deformity is minimal.
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This 71-year-old man presented with a dark lesion on his scalp. The lesion was first treated with an excisional biopsy performed by his local physician; the lesion recurred quickly. He was treated with wide excision and adjuvant radiation therapy. The specimen (hematoxylin and eosin, original magnification X12.5) shows a well-differentiated cutaneous angiosarcoma composed of irregular vascular channels.
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This young woman presented with multicentric angiosarcoma. The images show several bones of the right foot, the right distal femur, and the right patella affected with the process. The microphotograph of the specimen revealed a grade 2 angiosarcoma (hematoxylin and eosin, original magnification X100). Most bone tumors are solitary lesions with a very low incidence of multicentricity. Vascular tumors are an exception and may involve multiple bones. Angiosarcoma presents in this case as multiple lytic lesions in the same extremity.
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This is a soft tissue angiosarcoma presenting as a rapidly growing mass in the calf. This 69-year-old woman was treated with wide resection of the mass followed by external beam radiotherapy. The full-thickness graft obtained from the ipsilateral thigh at the moment of surgery aided in the closure and prevented further wound complications during the adjuvant radiotherapy. The rapid growth of soft tissue angiosarcomas explains why they are often misdiagnosed as abscesses and tentatively treated with drainage. The drainage is sanguinous, with blood clots, and hemostasis may be challenging. To avoid this problem, fine-needle aspiration should precede any attempt at incisional or excisional biopsy of a soft tissue mass.
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This 45-year-old man presented with progressive pain in his left hip. Activity-related at first, the pain turned constant. The patient described it as a dull ache and a boring sensation, with occasional stubbing episodes. The pelvic bone involvement was extensive. The patient was treated with an external hemipelvectomy. The patient survived for 1.5 years.
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Plain film of the tibia demonstrates small lucencies without internal matrix production in keeping with the diagnosis of a vascular lesion.
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Coronal T1 magnetic resonance imaging (MRI) study reveals a lesion of the diaphysis with cortical breakthrough.
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Coronal T2 magnetic resonance image shows a diaphyseal lesion with intense surrounding edema (high signal intensity).
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Axial T2 magnetic resonance image shows a heterogeneous signal lesion with cortical destruction.
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Grossly, angiosarcomas show a reddish-brown color and solid patterns.
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Foci of angiosarcomas can show well-formed vascular channels that aid in the diagnosis. These channels can be difficult to find in a large number of tumors.
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Atypical cells in solid sheets or nests are typical of this tumor and can make diagnosis on hematoxylin and eosin (H&E) sections difficult, necessitating the use of immunohistochemistry.
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CD34 immunohistochemistry highlights the vascular nature of angiosarcomas and aids in the diagnosis.
