Breast Angiosarcoma

“Sarcoma” is not a word that is generally associated with discussions of breast cancer. A sarcoma is actually a cancerous tumor of connective or muscle tissues (myoepithelial cells) while most breast tumors are ‘carcinoma‘ which is a tumor of cancerous lining and membrane cells, (epithelial cells) such as the lining of the breast ducts.

I just want to let you know that I have decided to create a newer version of this page with more up-to-date information on Angiosarcoma of the Breast. However, even though this page is getting a little bit out-dated and somewhat old, doesn’t mean you still can’t use it, because I totally would.

An ‘angio‘ sarcoma would indicate the involvement of blood supply elements in some way, and in fact an angiosarcoma is basically a cancer of the cells which line the blood vessels. An angiosarcoma could therefore develop in virtually any area of the body, but a breast angiosarcoma usually starts in the cells which line the blood vessels of the breast or the underarm area.

Breast angiosarcomas are relatively uncommon

Statistically, less than 10% of all angiosarcomas originate in the breast. In terms of breast cancers, angiosarcomas account for a very small percentage of cases; less than 0.05%, or approximately 1 in every 2500 breast cancers.

Breast angiosarcomas are also sometimes referred to by different names, such as mammary angiosarcoma, hemangiosarcoma of the breast, and lymphangiosarcoma if it develops in the area of the lymph nodes. Angiosarcomas of the breast account for about 8% of all breast sarcomas. Once they develop, an angiosarcoma of the breast tends to grow and spread rather quickly.

Primary and secondary breast angiosarcomas

A primary angiosarcoma is so termed because it develops in patients who have never had any history or treatments for breast cancer. A secondary angiosarcoma, on the other hand, is one that develops in a woman who has already had treatments for a primary breast cancer.

Primary angiosarcomas of the breast tend to occur in younger women, usually in the 20 to 40 years range, while a secondary breast angiosarcoma will tend to develop in women over 40, and can often develop 5 to 10 years after radiation treatments for breast cancer.

If fact, some breast cancer specialists consider secondary angiosarcoma of the breast entirely as a rare complication of breast radiation treatments. About 17% of patients develop breast angiosarcoma after breast radiation therapy for breast cancer.

Common clinical presentation of angiosarcoma of the breast

In the clinical setting, a primary breast angiosarcoma might simply feel like a ‘thickened‘ area of the breast, perhaps with a rash or the appearance of bruising. A secondary angiosarcoma of the breast will usually present clinically as a hard, painless breast mass. There may be some swelling and ‘bumpiness‘ to the mass, and the skin over the mass might be blue or reddish in color.

About 75%-80% of breast angiosarcomas present with a palpable mass, and approximately 16% will manifest with progressive breast swelling. But, 10% to 12% of breast angiosarcomas may not have any clinical symptoms at all. The average tumor size at time of diagnosis is often around 6 cm, and the average age at the time of diagnosis is around 40 years.

Mammographic features commonly associated with breast angiosarcomas

Both primary and secondary breast angiosarcomas will tend to appear on a mammogram as an ill-defined mass with a fuzzy outline, and without any of the ‘spikey‘ protrusions that one tends to find with invasive breast carcinomas. Some breast angiosarcomas are hidden to mammography, and this may be attributed in part to the dense breast parenchyma which one often finds in younger women.

In addition, breast angiosarcomas often appear to be vague, isodense, without calcifications, and without architectural distortions. In other words, some of the normal mammographic indicators of breast carcinoma are not present in angiosarcomas of the breast.

On ultrasound, a breast angiosarcoma will tend to be rather easy to see, with clear borders that may be ‘bumpy‘. Sonograms of breast angiosarcomas will usually appear as a mass that shows homogeneous or heterogeneous hyperechogenicity (with associated architectural distortion).

They will often have a hypervascular, hemorrhagic, and heterogeneous appearance. They usually appear as solitary, circumscribed, hypervascular masses with variable echo patterns and without posterior acoustic phenomena.

Of interest, the angular margins and posterior acoustic shadowing which are characteristic of breast carcinomas are not features of breast angiosarcoma ultrasounds. Breast carcinomas are rarely hyperechoic, but ultrasound images of angiosarcomas of the breast are hyperechoic or mixed hyper- and hypoechoic in about 50% of cases.

Breast MRI is occasionally used to diagnose angiosarcomas of the breast, which will be quite evident due to the involvement of the affected blood vessels in the tissues surrounding the tumor. With MRI, breast angiosarcomas tend to appear as large, lobular-shaped masses with indistinct borders, with a few tumors demonstrating infiltrative margins.

Typically, one may observe hemorrhagic areas and heterogeneous architecture. All breast angiosarcomas will be hypervascular and enhance intensely and heterogeneously, typically with rapid initial enhancement and washout kinetic features at dynamic imaging, which is characteristic of a malignant breast lesion.

Histological characteristics and grading of breast angiosarcomas

Generally speaking, breast angiosarcomas will show vasoformative features consisting of microacini, arborizing micro tissue fragments and intracytoplasmic lumen against a bloody background. If this is the case, the pathologist will certain suspect breast angiosarcoma, which will be confirmed by certain markers present in the immunohistochemical evaluation.

Breast angiosarcomas tend to be highly cellular and often composed of spindle to ovoid cells, often in loose cohesive clusters, sheets, and elongated cords. Quite often there are numerous single cells with a scanty pale blue cytoplasm. Nuclei in breast angiosarcoma cells tend to be either round, oval, or spindle shaped. Nucleoli are inconspicuous, and nuclear membranes tend to be smooth. Mitoses is usually not present.

Breast angiosarcomas are graded based on infiltration of vascular differentiated cells

Angiosarcomas of the breast are largely ‘graded‘ for staging purposes based on the histological evaluation of tumor cells. Generally speaking, grading is based upon the degree to which malignant cells exhibiting vascular differentiation have infiltrated the breast parenchyma.

Low-grade breast angiosarcomas tend to show prominent and freely anastomosing vascular channels. Endothelial ‘tufting‘ and papillary growth patterns are minimal or absent altogether. Low grade angiosarcomas of the breast also demonstrate very little cytological atypia.

An intermediate grade breast angiosarcoma will tend also to have freely anastomosing vascular channels, but will demonstrate a certain among of papillary growth and endothelial tufting. Intermediate grade angiosarcomas of the breast may also demonstrate focal solid areas of malignant cell growth with polygonal or spindle cells.

Breast angiosarcomas classified as high-grade will typically have prominent areas of cells which are clearly malignant, and often polygonal of spindled in appearance. ‘Lakes‘ of blood and evidence of necrosis are very common. In high grade breast angiosarcomas the vascular channels may be difficult to identify; the vascular nature of the tumor might only be evident through immunohistological analysis. The endothelial marker CD34 would be a strong indicator of breast angiosarcoma.

Treatment and prognosis for angiosarcoma of the breast

The ‘staging‘ criteria for breast angiosarcoma is not the same as for breast carcinomas, and in fact, breast angiosarcomas do not correspond well to the staging classifications for sarcomas in other areas of the body. The growth pattern or grade is an important prognostic factor with angiosarcomas of the breast.

On average, about 40% of cases of breast angiosarcoma are of a low grade, about 20% of an intermediate grade, and about 40% of a high grade.

Usually treated by surgery

Angiosarcoma of the breast is usually treated either by a wide local excision or by mastectomy. Some angiosarcomas are treated with adjuvant chemotherapy, but the effectiveness of chemical interventions varies widely. Small, frequent treatments by radiation therapy have been shown to have some effectiveness in preventing local recurrence of breast angiosarcomas.

Anti-angiogenesis therapy is in the early stages of clinical trial, and other experimental treatments by hyperthermia (heat), and immune system therapies have shown some promise. The size of the primary tumor does not really seem to influence prognosis for breast angiosarcomas, nor the length of time prior to the commencement of treatment.

Chemotherapy is used on average in about 50% of cases, but local recurrences of breast angiosarcomas seem to occur about 50% of the time whether chemotherapy is used or not.

Variable prognosis for breast angiosarcoma

The prognosis for breast angiosarcomas is variable, but it is a very serious diagnosis, especially if the tumor is of a high grade. It may be estimated that about 1/3 of women who develop breast angiosarcoma may not survive beyond three years following diagnosis, mostly due to metastasis of the disease to other areas of the body.

About 11-12% of women with breast angiosarcoma may develop local recurrence after 2 to 7 years, and in about 8% of women this may occur in the contralateral breast. However, about 50% of women with angiosarcoma of the breast remain healthy and disease free after three years.

The survival rate for low grade breast angiosarcomas is high

The five year disease free survival rate for low grade tumors can be as high as 76%, and up to 70% for intermediate grade tumors. However, the five year survival rate for high grade breast angiosarcomas is only about 15%.

In terms of lifetime survival with a disease free status, women with a low grade breast angiosarcoma will on average survive more than 15 years following diagnosis and treatment, and a woman with an intermediate grade tumor, properly treated, will still on average survive disease free for 12 more years. Unfortunately, high grade breast angiosarcomas are historically very aggressive and on average most patients do not survive beyond 15 months following diagnosis.

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For further reading, I suggest you go to this page, which has some information about leiomyosarcoma of the breast.

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