Angiosarcoma or AS is a very rare form of soft tissue tumour where malignant cells occur in the inner lining of the blood vessels and lymph vessels. The lymph vessels are an integral part of the immune system which assists in collecting bacteria, viruses and waste products from different parts of the body and eliminating them.
Since the body has blood and vessels throughout, this type of cancer can occur at any place in the body. But in most cases, Angiosarcoma occurs in the skin on the head and neck. In very rare case scenarios, it may shape up in the skin on other parts of the body, such as the breast. Malignant cells may also arise in deeper tissues, such as the liver and the heart. Being a type of soft tissue sarcoma, which majorly represent 1% of all solid malignancies or tumours, angiosarcomas in itself exemplify only about 1% of all soft tissue sarcomas.
Just like any other form of sarcoma, the exact cause of Angiosarcoma is yet unknown, but recent researches showcase that it chiefly happens when there is a change or mutation in the genetic matter or DNA of the blood vessel and lymph node cells. A cell's DNA includes the instructions that tell the cell what to do. These changes in the DNA instruct the cell to grow abnormally in size and number without dying. And ultimately the unstable cells amass in large numbers to form a clump of tissue or tumour within the organ. They can even break away from the original site and invade near and distant tissues and organs to spread the infection.
Certain risk factors that increase the risk of Angiosarcoma include:
Age: Angiosarcoma is more common in people aged 60 or older
Gender: Males have a higher incidence of developing primary AS than their female counterpart
Radiation Therapy: People undergoing or those who have undergone treatment with radiation for one or other form of cancer have a higher risk of Angiosarcoma
Health Condition: Suffering from lymphedema which characterizes swelling caused due to backup of lymph fluid may aggravate the chance of AS. Lymphedema can occur when the lymphatic system gets blocked or damaged, or when lymph nodes are removed during surgery or due to an infection or other conditions like swollen lymph nodes
Exposure to chemicals: Susceptibility to chemicals like polyvinyl chloride, arsenic and thorium dioxide may increase the risk of liver angiosarcoma
Genetic syndromes: Certain gene changes that children are diagnosed with at birth can raise the risk of having Angiosarcoma later in life. Some of the genetic conditions that might lead to Angiosarcoma include Maffucci syndrome, or Klippel- syndrome, Bilateral retinoblastoma, Ollier disease, Von Hippel–Lindau disease, Von Recklinghausen’s neurofibromatosis, and the BRCA1 and BRCA2 genes.
The characteristic signs and symptoms of Angiosarcoma may vary depending upon the site of cancer. Since it mostly occurs on the skin of the head and the neck, some of the common symptoms include:
AS In Skin Of Head And Neck
- A raised portion of the skin that looks like a bruise
- A bruise-like lesion that thrives larger gradually
- A lesion that may ooze out blood when scratched or bumped
- Swelling and inflammation in the skin surrounding the lesion
When angiosarcoma affects other deeper tissues and organs, such as the liver or the heart, the characteristic symptoms usually take time to crop up and sometimes, it simply causes pain.
AS In Liver
- Pain in the abdomen
AS In Breast
- Skin changes on the breast
- Purple-coloured nodules on the breast
- The appearance of lumps from within the breast tissue
Diagnosis And Treatment
On recognizing any of the above-mentioned signs and symptoms, do discuss with a specialised doctor right away to initiate the treatment at the earliest. The doctor usually does a thorough physical check-up followed by affirming the patient’s past medical history and genetic history and carries out a few of the diagnostics including:
Imaging tests: These include MRI-scan, CT-scan, PET-scan (Positron Emission Tomography) etc.
Biopsy: Examining a mass of tissue that is peeled off from the affected body part to look for cancer cells and the appropriate stage of the infection
The available treatment options for Angiosarcoma usually depend upon the age of the person, size and stage of the tumour, and whether it has spread to other parts of the body. These include:
- Radiation therapy
- Targeted-Drug therapy
- Clinical Trials
- Supportive care