Angiosarcoma

Angiosarcoma is an uncommon but highly aggressive cancer that originates in the endothelial cells lining blood vessels and lymphatic vessels. This malignancy falls under the umbrella of soft tissue sarcomas, which develop in connective tissues of the body. Unlike more common skin cancers, angiosarcoma can arise anywhere in the body where blood or lymph vessels exist, including the skin, liver, spleen, heart, and breast.

Due to its rarity and rapid progression, angiosarcoma poses unique challenges for diagnosis and treatment. It is often mistaken for benign conditions such as bruises or infections in its early stages, leading to delayed diagnosis. Early detection is crucial to improve treatment outcomes and survival chances.

This article aims to provide an in-depth understanding of angiosarcoma, from its causes and symptoms to treatment options and living with the disease, with special attention to the prevalence in India and worldwide.

Angiosarcoma is a malignant tumour that arises from the endothelial cells forming the walls of blood vessels and lymphatic vessels. Because these cells are integral to the circulatory and lymphatic systems, tumours can spread quickly through the bloodstream or lymphatic system to distant organs.

These tumours are characterised by rapid growth, high invasiveness, and a tendency to metastasise early. Angiosarcomas can be either cutaneous (affecting the skin), visceral (affecting internal organs), or associated with chronic conditions like lymphoedema.

Despite their aggressive nature, angiosarcomas are relatively rare, accounting for approximately 1-2% of all soft tissue sarcomas globally.

Angiosarcoma is a rare cancer worldwide, with an estimated incidence of 1 to 2 cases per million people annually. It constitutes about 1-2% of all soft tissue sarcomas.

In India, accurate prevalence data is limited due to a lack of comprehensive cancer registries, but it is believed to be similarly rare. The limited awareness and diagnostic challenges contribute to underreporting.

The rarity of angiosarcoma means that many clinicians may only encounter a few cases in their careers, emphasising the importance of specialised cancer centres for management.

Angiosarcoma is categorised based on the tumour’s primary location or associated risk factors:

1. Cutaneous Angiosarcoma:
   The most common form, often appearing on the scalp, face, and neck of elderly patients. It usually starts as purplish bruise-like patches that expand and can ulcerate.
2. Lymphoedema-associated Angiosarcoma (Stewart-Treves Syndrome):
   Develops in areas with chronic lymphoedema, often in the arm following lymph node removal during breast cancer surgery. The chronic swelling creates an environment conducive to malignant transformation.
3. Radiation-induced Angiosarcoma:
   Arises in previously irradiated tissues, commonly after radiotherapy for breast or other cancers. This form may develop years after radiation exposure.
4. Visceral Angiosarcoma:
   Occurs in internal organs such as the liver, spleen, heart, or breast. Hepatic angiosarcoma is linked to certain chemical exposures and has a very poor prognosis.
5. Primary Breast Angiosarcoma:
   A rare form that affects breast tissue without previous radiation or lymphoedema.

Each subtype varies in clinical presentation and treatment approach, making accurate classification essential for management.

The precise cause of angiosarcoma remains uncertain, but several risk factors have been identified:

• Chronic lymphoedema:
  Prolonged swelling due to lymphatic obstruction (e.g., after mastectomy) is strongly linked to lymphangiosarcoma development.
• Radiation exposure:
  Previous therapeutic radiation increases the risk, especially in breast cancer survivors.
• Chemical exposure:
  Industrial chemicals such as vinyl chloride, arsenic, and thorium dioxide are associated with hepatic angiosarcoma.
• Genetic mutations:
  Alterations in oncogenes and tumour suppressor genes (e.g., TP53) may contribute.
• Age and gender:
  Most cases occur in older adults, with some male predominance in specific types.
• Immunosuppression:
  Conditions suppressing immune surveillance may increase susceptibility.

Environmental and occupational exposures, alongside genetic susceptibility, create a complex web of causation that researchers continue to explore.

Symptoms vary widely depending on tumour location but often include:

• Skin Angiosarcoma:
  Appears as purplish or bruise-like patches or nodules that progressively enlarge. These lesions may be tender, bleed easily, or ulcerate. They commonly appear on the scalp, face, or limbs.
• Lymphoedema-associated:
  Persistent swelling with red or purple skin discolouration.
• Visceral Angiosarcoma:
  Symptoms relate to organ dysfunction, such as abdominal pain, jaundice, or unexplained weight loss (liver involvement), or shortness of breath and chest pain (lung involvement).
• General signs:
  Fatigue, night sweats, and fever in advanced disease.

Because symptoms often mimic benign conditions, they are frequently overlooked, delaying diagnosis.

Accurate diagnosis requires a combination of clinical suspicion and specialised investigations:

• Physical examination:
  Evaluation of suspicious skin lesions or palpable masses.
• Imaging studies:
  MRI and CT scans provide information on tumour size, depth, and spread. PET scans may detect metastases.
• Biopsy:
  The gold standard for diagnosis; tissue samples are examined microscopically.
• Histopathology:
  Reveals irregular, abnormal blood vessel formation with atypical endothelial cells.
• Immunohistochemistry:
  Positive staining for endothelial markers (CD31, CD34, Factor VIII) confirms vascular origin.
• Laboratory tests:
  May assess overall health and organ function, particularly in visceral disease.

Early biopsy of suspicious lesions is essential to avoid diagnostic delays.

Treatment typically involves a multidisciplinary team and is personalised based on tumour type, size, location, and stage:

1. Surgery:
   Primary treatment for localised tumours, aiming for complete excision with clear margins. Wide local excision is often necessary.
2. Radiation therapy:
   Used pre- or post-operatively to reduce recurrence risk or treat unresectable tumours.
3. Chemotherapy:
   Agents like paclitaxel, doxorubicin, and ifosfamide are standard, especially in advanced or metastatic cases. Chemotherapy may be combined with radiation. (BRAND: Bucelon 60 mg)
4. Targeted therapies:
   Emerging treatments targeting vascular endothelial growth factor (VEGF) and other pathways show promise.
5. Immunotherapy:
   Currently under research, with some trials investigating checkpoint inhibitors.

Treatment aims not only at controlling cancer but also at preserving function and quality of life.

While no alternative therapy cures angiosarcoma, some complementary approaches can support symptom management and wellbeing:

• Pain relief:
  Techniques like acupuncture and massage may alleviate discomfort.
• Nutritional support:
  Balanced diets help maintain strength during treatment.
• Psychological support:
  Counselling and support groups assist with emotional challenges.

Always discuss alternative therapies with your oncology team to ensure they do not interfere with conventional treatment.

Known risk factors include:

• Previous radiation treatment.
• Chronic lymphoedema.
• Exposure to carcinogenic chemicals.
• Older age.
• Genetic predisposition.
• Immunosuppression or autoimmune conditions.

Understanding these factors can help identify individuals who require closer monitoring.

Potential complications arise from the disease or its treatment:

• Local tissue destruction and ulceration.
• Spread to lymph nodes and distant organs.
• Secondary infections from open wounds.
• Side effects of chemotherapy and radiation (e.g., neuropathy, marrow suppression).
• Psychological distress and reduced quality of life.

Timely management and supportive care reduce the risk of serious complications.

• Adhere strictly to treatment plans and follow-up schedules.
• Maintain a nutritious diet and stay hydrated.
• Manage pain with professional guidance.
• Seek emotional and social support.
• Avoid smoking and excessive alcohol consumption.
• Rest adequately and engage in gentle physical activity as tolerated.

Maintaining a positive outlook and strong support system helps improve quality of life.

1. Angiosarcoma Is Not Just Skin Cancer

Many believe angiosarcoma only affects the skin, but it can develop anywhere in the body where blood or lymph vessels are present — including the heart, liver, and breast. It’s a vascular cancer, not a typical skin cancer.

2. It’s Rare but Extremely Aggressive

Contrary to the myth that it grows slowly, angiosarcoma is highly aggressive and spreads rapidly. Early symptoms may look like bruises or swelling, leading to misdiagnosis or delayed treatment. Timely diagnosis is crucial for better outcomes.

3. Standard Treatments Are Essential – Not Optional

There’s a misconception that alternative therapies can cure angiosarcoma, but evidence-based treatments like surgery, chemotherapy, and radiation are necessary. While supportive therapies may help manage symptoms, they cannot replace medical care.

Seek medical attention if you notice:

• Persistent skin changes that resemble bruises
• Painless lumps or swelling
• Unexplained weight loss or fatigue
• Shortness of breath or chest pain
• Any recurring symptoms post-cancer treatment

• What type of angiosarcoma do I have?
• What stage is my cancer?
• What are my treatment options?
• What side effects can I expect?
• How will treatment affect my daily life?
• Are there clinical trials available for me?

• Offer Emotional Support: Be a good listener.
• Assist Practically: Help with hospital visits and daily tasks.
• Respect Privacy: Allow them space to cope.
• Educate Yourself: Learn about the condition.
• Encourage Positivity: Celebrate small victories.

Angiosarcoma is a rare, aggressive cancer with complex diagnosis and treatment challenges. Awareness of risk factors, early symptoms, and the importance of prompt medical attention can improve outcomes. Advances in chemotherapy, radiation, and targeted therapies offer hope, but multidisciplinary care remains essential. Patients benefit greatly from comprehensive support addressing physical and emotional needs.