What is Epithelioid angiosarcoma?
Epithelioid angiosarcoma is a rare type of angiosarcoma. The epithelioid angiosarcoma is a malignant cancer of endothelial cells. It arises in the soft tissues, but primary sites such as thyroid, skin, adrenals, and bone are also encountered. Angiosarcoma is the malignant neoplasm of the mesenchymal cell in which cancer cells exhibit endothelial differentiation.
The Angiosarcomas have a poor prognosis because they have a high tendency of recurrence and metastases. Metastasis is a process by which the cancer cells spread to other parts of the body. The cancer cell may detach to its primary tumour and start invading the nearby organs, lymph nodes, and tissues. This gives rise to secondary tumours or cancer. The metastases of cancer to the other region are based entirely on the location of the primary tumour or cancer and its nature, whether benign or malignant. Such types of cancers are usually called stage 4 cancers and are usually named based on the organ or tissue from which they originate.
Like Epithelioid angiosarcoma, stage 4 arises in the soft tissues but metastasizes to the thyroid, adrenal glands, and bone. Recurrence refers to the reappearing of cancer after the treatment. However, it takes some time to reappear. Local recurrence refers to the appearance of the tumour on the primary site. Seasonal recurrence refers to the appearance of cancer in the surrounding region of the initial site. Distant recurrence refers to th
e appearance of cancer in the body parts away from the primary site. Recurrence usually happens within two years. The risk factors associated with the recurrence are incomplete excision, delayed diagnosis, and primary disease extension.
Epithelioid angiosarcoma stage 4 Symptoms
The symptoms of angiosarcoma differ depending upon the site of the tumour. The angiosarcoma may present itself in different forms. It may appear as a non-healing skin lesion, infection, or wound. It can also appear as a soft palpable lump that can be seen. The deep-rooted tumours do not show any sign until they start invading other organs. In 50% of the patient, cancer is already spread in the body from its primary or original tumour at the time of diagnosis. Angiosarcomas have a tendency for local recurrence, wide-spreading, and high chances of systemic and lymph node metastases. They are slow-growing tumours.
The most common sites of epithelioid angiosarcoma are the upper and lower extremities. They often present themselves as firm-to hard masses that can be felt and seen. These masses are either in the dermis or the deep soft tissues. Sometimes they represent themselves as non-healing superficial wounds or lesions. The deep-rooted tumour is often mistaken for ganglion cyst or giant cell tendon sheath tumours because they attach themselves to the tendons. The symptoms with which patients often present are pain, tenderness, weakness, hypoesthesia, multifocal tumour, and metastases. Epithelioid angiosarcoma has the tendency to cause metastases of the lymph node, lung, and lung. The chemotherapy, radiotherapy, and surgery are done for small tumours in angiosarcoma patients.
Epithelioid angiosarcoma stage 4 causes
The cause of Epithelioid angiosarcoma is unknown in the majority of the cases. Some of the causes of the Epithelioid angiosarcoma are given below:
· Genetics
· Lymphedema, the accumulation of lymph fluid in the tissue which leads to swelling
· Exposure to radiation or past radiation therapy
· Exposure to carcinogens such as arsenic
Epithelioid angiosarcoma stage 4 survival rate
The patients with Epithelioid angiosarcoma are often diagnosed when the tumour has already spread all over their body. This late diagnosis leads to a poor prognosis and reduces the survival rate. The chances of better prognosis and increased survival rate can be achieved when the tumours are small and present at such regions where they can be removed easily and with clear margins.
The low-grade breast angiosarcoma has a better prognosis, whereas the worst prognosis can be seen with the soft tissue angiosarcomas because the examination of soft tissue sarcomas is very difficult. The factors such as sex, location of the tumour, age, tumour size, mitotic index, and microscopic pathology affect the prognosis of epithelioid angiosarcoma. Based on sex, females show better outcomes and prognosis than males. Based on the tumour or lesions’ location, the distal lesions have a better prognosis and outcome than the proximal lesions. Age also affects the prognosis; the patients who are diagnosed at an early age have a better prognosis than the people with old age. The worst outcome is seen with the tumours that undergo necrosis and vascular invasion.
The angiosarcomas have a high tendency of local recurrence and metastasis because of their intrinsic biological properties. Angiosarcomas are very aggressive with rapid proliferation and are difficult to treat, therefore, have a high mortality rate and poor prognosis. The five-year survival rate in the Epithelioid angiosarcoma patient is 50-70%, whereas the ten-year survival rate in the Epithelioid angiosarcoma patient is 42-55%. The angiosarcoma patient should be monitored even after the treatment and removal of cancer because of its tendency of recurrence. For the first two years, after every three months follows up should be made. Later, after every six months.