Angiosarcoma Clinical Presentation

Updated: Jul 12, 2018
  • Author: Belen Carsi, MD, PhD, FRCS; Chief Editor: Edwin Choy, MD, PhD  more...
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Presentation

History

Angiosarcomas are insidious, and they may not produce symptoms until the disease is well advanced. [13] History should focus on identifying risk factors; however, most patients do not have these factors.

Risk factors are as follows:

  • Toxic exposure or radiation therapy
  • Other carcinogens (eg, bone wax, Dacron, metal bodies)
  • Lymphedema

Angiosarcomas arising at different sites and in different organs have some distinct features, but the clinical manifestations they cause are associated with the amount of organ tissue replaced, as follows:

  • Pathologic fractures, anemia, or hepatic dysfunction

  • Other intrinsic characteristics of a malignant vascular proliferation (eg, bleeding, thrombocytopenia, or disseminated intravascular coagulation)

  • Compression of adjacent neurovascular structures that causes pain

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Causes

The etiology of most cases of angiosarcoma is unknown. The tumors may develop as a complication of a preexisting condition. The following factors may be associated with tumor development [14, 11] :

  • Radical mastectomy
  • Radiotherapy
  • Foreign materials
  • Environmental carcinogens
  • Preexisting benign lesions (eg, bone infarct, pagetoid bone, chronic osteomyelitis)

Radical mastectomy

Chronic lymphedema is the most widely recognized risk factor, especially in angiosarcomas of the skin and soft tissue. Typically, lymphedema-associated angiosarcomas occur in women who have undergone radical mastectomy for breast carcinoma and have had chronic lymphedema for many years (Stewart-Treves syndrome) or in the leg of patients as a consequence of radical inguinal lymphadenectomy for metastases from malignant melanoma (Kettles syndrome).

Chronic lymphedema occurring on a congenital, idiopathic, traumatic, or infectious basis also predisposes to angiosarcoma. The rationale for this association is the status of immunologic privilege of a lymphedematous region.

Radiotherapy

Radiation-induced angiosarcomas occur in the absence of chronic lymphedema after radiotherapy for carcinoma of the cervix, ovary, endometrium, or breast and Hodgkin disease.

The lesion arises in the area of previous radiation, with an interval between irradiation and the development of the new tumor of approximately 10 years. The risk of postradiotherapy sarcomas appears to augment with increasing dosage.

The diagnosis mandates that the lesion must have proven histologic differences from the primary neoplasm (carcinomas, lymphomas). Angiosarcomas of bone arising in a previously radiated bone are third in frequency after osteosarcoma and fibrosarcoma. Angiosarcoma of soft tissue is the first diagnosis in soft tissue sarcomas arising within the field of radiation, followed by malignant fibrous histiocytoma (MFH).

The Finnish Cancer registry [15] suggests that although an increased risk of angiosarcoma in cancer patients is evident, especially with breast [16] and gynecologic cancer, the excess does not appear to be strongly related to radiotherapy. Contrary to this finding, other researchers suggest that adjuvant radiotherapy increases the risk of breast angiosarcoma 9-fold.  Regardless, radiation-associated angiosarcoma of the breast is rare, occurring in approximately 0.9 of 1,000 cases. Reported onset is as late as 23 years following radiotherapy. [17]

Foreign materials

Some angiosarcomas are associated with foreign material introduced in the body, either iatrogenically or accidentally. This association is described with the following materials:

  • Dacron
  • Shrapnel
  • Steel
  • Plastic graft material
  • Surgical sponges
  • Bone wax

Environmental carcinogens

Vintners who spray vines against mildew and patients with psoriases given prolonged treatment with Fowler solution (1% potassium arsenite) are at risk. Exposure to arsenic may increase the risk of angiosarcoma of the liver.

Dioxin, a contaminant of industrial processes, is a controversial risk factor associated with the development of soft tissue sarcomas. Likewise, exposure to vinyl chloride used in polymerization in the plastic industry can lead to angiosarcomas of liver and soft tissue.

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Physical Examination

Physical examination findings often are unremarkable; however, subtle findings may provide clues to early detection.

Angiosarcoma of soft tissue

Patients with soft-tissue angiosarcomas [6, 12] usually present with a moderately paced growing mass in the extremities; the rapid progression of the disease is sometimes the clue to the diagnosis. Soft-tissue angiosarcomas may also develop in the retroperitoneum or abdominal wall.

Retroperitoneal angiosarcomas usually present as asymptomatic masses and generally grow to large sizes because the abdomen can accommodate tumors. Patients may present with neurologic symptoms from compression of lumbar or pelvic nerves.

Approximately 33% of patients have evidence of recent hemorrhage or coagulopathy, including anemia, persistent hematoma, hemothorax, hemorrhagic ascites, and gastrointestinal bleeding. Frequently, the adjacent nodes are enlarged; the incidence rate of node metastasis is as high as 45%

Angiosarcoma of bone

Angiosarcoma of bone [18] can affect any portion of the skeleton, although 33% of these tumors occur in the axial skeleton, 33% in long tubular bones, and the rest in the small bones of the hands and feet. These tumors can be multifocal, affecting the same bone with multiple lesions, or multicentric, involving multiple bones of the same extremity. The patients do not present specific symptoms, although pain is common and the area is frequently tender.

Sometimes, swelling and increased size of the affected limb due to affectation of a superficial bone or to soft tissue extension characterize the presentation. Pathologic fractures occur in 10% of patients.

Cutaneous angiosarcoma

Four variants of cutaneous angiosarcoma are currently recognized, as follows [19, 14] :

  • Angiosarcoma of the scalp and face

  • Angiosarcoma in the context of lymphedema (Stewart-Treves syndrome)

  • Radiation-induced angiosarcoma

  • Epithelioid angiosarcoma

Angiosarcoma of the scalp and face

This is the most common form of angiosarcoma. The disease is primarily located on the head and neck, and usually occurs in elderly persons; it is also known as Wilson-Jones angiosarcoma, senile angiosarcoma, or malignant angioendothelioma.

Most patients present with an enlarging bruise, a blue-black nodule, or an unhealed ulceration. Initially, these lesions can be confused with cellulitis, edema, bruising, or infection, leading to a delay in diagnosis. Bleeding and pain may be present. The clinical pattern of the lesions may be nodular, diffuse, or ulcerated.

Cutaneous angiosarcoma associated with lymphedema

Lymphedema -associated angiosarcoma (LAS) was first reported in six patients with postmastectomy lymphedema. In each case, angiosarcoma developed in the ipsilateral arm and occurred several years after mastectomy. Subsequently, LAS was reported after axillary node dissection for melanoma and in the context of congenital lymphedema, filarial lymphedema, and chronic idiopathic lymphedema. The risk for developing LAS 5 years after mastectomy is approximately 5%. The most common site is the medial aspect of the upper arm. LAS presents as a violaceous plaque or nodule superimposed on brawny, nonpitting edema. Ulceration may develop rapidly.

Radiation-induced angiosarcoma

These lesions occur in the radiation field 4-40 years after irradiation. Exposure to Thorotrast (a radioactive radiographic contrast agent used in the United States until the 1950s) may lead many years later to liver angiosarcoma.

Epithelioid angiosarcoma

This is a rare, recently described variant with an aggressive course. Death occurs 2-3 years after presentation.

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