What is a Primary Splenic Angiosarcoma?
Primary splenic angiosarcoma is a very rare and highly aggressive cancer of the spleen. The spleen is an organ located in the upper left side of the abdomen and plays an important role in filtering blood and supporting the immune system.
In this disease, the cancer starts from the cells that line the blood vessels of the spleen, causing them to grow in an abnormal and uncontrolled manner.
Incidence
This cancer mostly affects older adults and is slightly more common in men. In many cases, there is no clear cause, although exposure to certain chemicals and radiation has been suggested in a few reports. Because primary splenic angiosarcoma is extremely rare and its early symptoms are vague, it is often diagnosed late, when the disease has already progressed.
Symptoms
The symptoms develop gradually and may be mistaken for common abdominal or blood-related problems. People often experience pain or heaviness in the upper left side of the abdomen, which may spread to the left shoulder. The spleen may become enlarged, leading to abdominal swelling or a feeling of fullness even after eating small amounts.
Many patients feel extreme tiredness and weakness due to anemia caused by internal bleeding. Fever, weight loss, and loss of appetite are also common. One of the most dangerous complications is sudden rupture of the spleen, which can cause severe internal bleeding, sudden abdominal pain, low blood pressure, and can be life-threatening.
Diagnosis
Diagnosis is made using imaging tests such as ultrasound, CT scan, or MRI, which may show an enlarged or abnormal spleen. However, imaging alone cannot confirm the diagnosis. A definite diagnosis is usually made after removal of the spleen, when the tissue is examined under the microscope and special tests confirm the blood vessel origin of the cancer.
Treatment
The main treatment is surgical removal of the spleen, especially if the tumour is detected before rupture occurs. Surgery can help control symptoms and prevent life-threatening bleeding. Unfortunately, even after surgery, this cancer has a strong tendency to spread to other organs, particularly the liver, lungs, and bones. Chemotherapy may be used after surgery, but its benefit is limited.
Prognosis
The prognosis is generally poor because the cancer is very aggressive and often spreads early. Many patients have a short survival time, although early detection and prompt surgery can slightly improve outcomes.
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