Angiosarcoma: Symptoms, Causes, Diagnosis, Treatment, and Survival Outlook

Angiosarcoma is a rare, aggressive form of cancer that develops from the cells lining blood vessels or lymph vessels. It belongs to the broader group of soft tissue sarcomas and is known for its rapid growth, early spread, and high recurrence rate. Because symptoms often mimic benign conditions, angiosarcoma is frequently diagnosed at an advanced stage.

This in-depth guide covers everything you need to know about angiosarcoma symptoms, causes, types, diagnosis, treatment options, prognosis, survival rates, and ongoing research.

~What Is Angiosarcoma?

Angiosarcoma is a malignant tumor arising from endothelial cells, which form the inner lining of blood and lymphatic vessels. These tumors can develop anywhere in the body but are most commonly found in the skin, breast, liver, spleen, and deep soft tissues.

Angiosarcoma accounts for less than 2% of all soft tissue sarcomas, making it extremely rare but highly aggressive.

~Types of Angiosarcoma

Angiosarcoma is classified based on its location and underlying cause.

1. Cutaneous Angiosarcoma

• Most common form

• Usually affects the scalp, face, and neck, especially in older adults

• Often appears as a bruise-like or purplish skin lesion

2. Breast Angiosarcoma

• Can be primary (arising in breast tissue) or secondary

• Secondary breast angiosarcoma is often linked to radiation therapy for breast cancer

3. Hepatic (Liver) Angiosarcoma

• Rare but highly aggressive

• Associated with exposure to vinyl chloride, arsenic, or thorium dioxide

4. Cardiac Angiosarcoma

• Usually affects the right atrium of the heart

• Often diagnosed late due to nonspecific symptoms

5. Lymphedema-Associated Angiosarcoma (Stewart–Treves Syndrome)

• Develops in areas of chronic lymphedema

• Commonly seen in arms after mastectomy and lymph node removal

~Causes and Risk Factors of Angiosarcoma

The exact cause of angiosarcoma is not fully understood, but several risk factors have been identified.

Known Risk Factors

• Radiation therapy (especially years after treatment)

• Chronic lymphedema

• Chemical exposure:

  • Vinyl chloride

  • Arsenic

  • Thorium dioxide

• Advanced age

• Male gender (slightly higher risk)

• Genetic mutations affecting endothelial cells

Most cases occur sporadically, without a clear inherited cause.

~Symptoms of Angiosarcoma

Symptoms vary widely depending on the tumor’s location and stage. Early symptoms are often subtle, leading to delayed diagnosis.

Common Symptoms

• Rapidly growing mass

• Pain or tenderness

• Fatigue and weakness

• Unexplained weight loss

• Bleeding from tumor sites

Site-Specific Symptoms

Skin Angiosarcoma

• Bruise-like or purplish lesions

• Skin thickening or swelling

• Non-healing sores

• Bleeding from lesions

Breast Angiosarcoma

• Breast swelling

• Skin discoloration

• Lump or thickened area

• Pain or heaviness

Liver Angiosarcoma

• Abdominal pain

• Jaundice

• Enlarged liver

• Internal bleeding

Cardiac Angiosarcoma

• Shortness of breath

• Chest pain

• Heart rhythm disturbances

• Fluid around the heart (pericardial effusion)

~How Angiosarcoma Is Diagnosed

1. Clinical Examination

A detailed physical examination helps assess lesion appearance, size, and progression.

2. Imaging Studies

• MRI – preferred for soft tissue evaluation

• CT scan – detects metastasis and internal organ involvement

• PET scan – identifies cancer spread and activity

3. Biopsy

A core needle or excisional biopsy is required to confirm angiosarcoma.

4. Pathology and Immunohistochemistry

Angiosarcoma typically tests positive for:

• CD31

• CD34

• ERG

• Factor VIII–related antigen

~Staging of Angiosarcoma

Angiosarcoma is staged using the TNM system, considering:

• Tumor size and depth

• Lymph node involvement

• Distant metastasis

• Histologic grade

Stages

• Stage I: Localized, low-grade tumor

• Stage II–III: Larger, high-grade tumors or local spread

• Stage IV: Distant metastasis (lungs, liver, bones, brain)

~Treatment Options for Angiosarcoma

Angiosarcoma requires aggressive, multidisciplinary treatment due to its rapid progression.

1. Surgery

• Primary treatment for localized disease

• Goal: Wide surgical excision with clear margins

• Often challenging due to infiltrative growth

2. Radiation Therapy

• Used before or after surgery

• Can improve local control

• Also used for palliation in advanced disease

3. Chemotherapy

Angiosarcoma is more chemotherapy-sensitive than many other sarcomas.

Common drugs include:

• Paclitaxel

• Doxorubicin

• Ifosfamide

• Gemcitabine

• Docetaxel

Weekly paclitaxel is commonly used, especially for cutaneous angiosarcoma.

4. Targeted Therapy

• Pazopanib (VEGF inhibitor)

• Anti-angiogenic agents targeting blood vessel growth

5. Immunotherapy

Emerging evidence suggests some benefit from:

• Immune checkpoint inhibitors (PD-1/PD-L1 inhibitors)

• Clinical trials remain crucial

~Recurrence and Metastasis

Angiosarcoma has a high rate of local recurrence and distant metastasis.

Common Metastatic Sites

• Lungs

• Liver

• Bones

• Brain

• Lymph nodes

Regular follow-up imaging is essential for early detection.

~Prognosis and Survival Rates

Prognosis depends on:

• Tumor location

• Size and depth

• Surgical margins

• Presence of metastasis

• Response to therapy

Estimated 5-Year Survival Rates

• Localized disease: 35–50%

• Regional spread: 20–30%

• Metastatic disease: <15%

Cutaneous angiosarcoma of the scalp and face tends to have a poorer outcome.

~Living With Angiosarcoma

Follow-Up Care

• Imaging every 3–4 months initially

• Lifelong surveillance often required

Supportive Care

• Pain management

• Wound care for skin lesions

• Psychological counseling

• Nutrition and physical therapy

Patient Support

• Sarcoma support organizations

• Oncology social workers

• Palliative care teams when needed

~Ongoing Research and Clinical Trials

Current research focuses on:

• Angiogenesis inhibitors

• Immunotherapy combinations

• Molecular profiling for personalized treatment

• Novel drug delivery systems

Because angiosarcoma is rare, clinical trial participation is strongly encouraged when available.

~Prevention and Early Detection

There is no proven way to prevent angiosarcoma, but early diagnosis can improve outcomes.

Seek Medical Attention If:

• A bruise-like lesion grows or bleeds

• Persistent swelling in previously irradiated areas

• New mass in lymphedematous limbs

~Frequently Asked Questions (FAQs)

Is angiosarcoma curable?

Early-stage angiosarcoma may be controlled with aggressive treatment, but recurrence is common.

Is angiosarcoma hereditary?

Most cases are not inherited.

How fast does angiosarcoma spread?

Angiosarcoma is known for rapid growth and early metastasis.

Is angiosarcoma painful?

Pain varies depending on location and tumor size.

~Conclusion

Angiosarcoma is a rare but highly aggressive cancer that demands early recognition and expert care. Advances in chemotherapy, targeted therapy, and immunotherapy are gradually improving outcomes, but long-term survival remains challenging.

Prompt diagnosis, treatment at specialized sarcoma centers, and access to clinical trials are critical for improving both survival and quality of life.