From Lymphedema to Cancer: Do You Know How This Transformation Happens?

Lymphedema is a condition in which fluid accumulates in the tissues due to a malfunctioning lymphatic system. While the condition itself does not directly cause cancer, studies have shown that lymphedema may be a catalyst for certain types of cancer, particularly those of a vascular origin, such as angiosarcoma.

In some cases, long-standing lymphedema may predispose to the development of angiosarcoma, particularly in patients who have been affected by surgery or radiation therapy.

Angiosarcoma is a rare and extremely aggressive cancer that originates in the endothelial cells that line the blood or lymph vessels. These tumors can occur anywhere in the body and at any age, but older people are most often affected.

According to statistics, the skin is the most common site of occurrence of angiosarcoma, accounting for approximately 60% of cases. Specifically, cases of the scalp account for approximately 50% of angiosarcomas. Because the behavior of these tumors is variable, symptoms may range from no symptoms to nonspecific symptoms such as skin lesions, ulcers, shortness of breath, and abdominal pain.

Early diagnosis is often the key to survival; however, the multi-organ involvement of angiosarcoma makes diagnosis and treatment difficult.

The cause of angiosarcoma is unknown, but certain risk factors have been identified, including chronic lymphedema, radiation therapy, and various chemicals such as arsenic and vinyl chloride. Angiosarcoma cells arise from the endothelium of blood or lymph vessels, so they can easily spread to other parts of the body, especially the liver and lungs, increasing their lethality.

Although surgery, chemotherapy, and radiotherapy are common treatment options, they pose a huge challenge to patients as the prognosis is usually poor. The five-year survival rate for all types of angiosarcoma is only 30-38%, and the prognosis for angiosarcoma of the heart and liver is even worse, with a survival period of only three months.

Angiosarcomas make up only 1-2% of soft tissue sarcomas, and these sarcomas account for less than 1% of all cancers in adults.

In addition, there are multiple classifications of angiosarcoma depending on the site of origin and associated risk factors. For example, primary cutaneous angiosarcomas often have no obvious cause, whereas lymphedema-associated angiosarcomas often occur in patients who have undergone mastectomy.

Angiosarcoma is usually diagnosed by MRI, CT, or ultrasound. However, differentiation can be difficult with these imaging studies alone, and biopsy and subsequent histologic and immunohistochemical analysis are often required to confirm the diagnosis. For angiosarcoma, CD31 is not only one of the gold standard diagnostic markers, but also shows higher sensitivity and specificity compared with other cancer markers.

In addition to humans, animals such as dogs can also suffer from angiosarcoma, especially golden retrievers, boxers and Labradors are more susceptible to this disease. Angiosarcoma progresses very rapidly and can quickly metastasize to other organs, showing extremely high lethality.

Currently, as our understanding of angiosarcoma deepens, it provides us with some important insights and potential future research directions. These rare but deadly cancers undoubtedly need more attention and research in order to better understand their pathological mechanisms.

In the future, how can we effectively increase cancer screening awareness among patients with lymphedema to improve the chances of early detection of pathological changes?

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