Purple-red spots on skin: Could this be deadly angiosarcoma?

The appearance of purple-red spots on the skin may make people feel uneasy. This phenomenon may be one of the manifestations of angiosarcoma, a rare and aggressive cancer. Angiosarcoma is a cancer that originates in the endothelial cells of blood or lymphatic vessels and can appear at any age, but is particularly common in older adults. According to statistics, the skin accounts for approximately 60% of all angiosarcoma cases, while the scalp region accounts for approximately 50% of these cases.

During the diagnosis process, angiosarcoma may be misdiagnosed as a benign tumor, resulting in delays in correct diagnosis and treatment.

Symptoms of angiosarcoma can range from completely asymptomatic to vague symptoms such as skin lesions, ulcers, dyspnea, and abdominal pain. When a diagnosis is confirmed, multiple organs are often involved, making identification of its origin and treatment options difficult. Although the exact cause of angiosarcoma is not fully understood, some risk factors such as chronic lymphedema, radiation therapy, and some chemicals (such as arsenic and vinyl chloride) are known factors.

This cancer may also develop in the long-term presence of foreign objects and is occasionally associated with breast implants. Ultraviolet radiation and local immunodeficiency may influence its pathogenesis. Imaging studies such as MRI, CT, and ultrasound can help visualize angiosarcoma, but differential diagnosis from other cancers is difficult, and biopsy and immunohistochemistry analysis are often required to confirm the diagnosis.

The prognosis of angiosarcomas is generally poor, with five-year survival rates of only 30–38% even with treatment, and angiosarcomas of the heart and liver lower the prognosis to only three months of survival.

Angiosarcomas make up 1-2% of all soft tissue sarcomas, and these sarcomas account for less than 1% of adult cancers. There are not many studies on this disease, and most of them are only case reports of small samples. However, the accumulated data have provided enough information for us to have a basic understanding of this disease. In recent years, the incidence of angiosarcoma has shown signs of increasing in the United States.

Classification of angiosarcoma

Angiosarcomas are classified based on their origin and underlying risk factors. Here are some of the main categories:

Primary cutaneous angiosarcoma

Refers to angiosarcoma in the skin without obvious underlying risk factors such as lymphedema or radiation therapy, mainly occurring on the head or neck, especially the scalp.

Lymphedema-associated angiosarcoma

Also known as Stewart-Treiff syndrome, it is mainly related to chronic lymphedema, which often occurs in the breast. After a mastectomy, lymph nodes are removed that may cause this condition.

Substantial organ angiosarcoma

This type of angiosarcoma occurs in solid organs such as the liver, breast, or heart, and accounts for approximately 40% of angiosarcoma cases.

Primary breast angiosarcoma

Breast angiosarcoma without obvious cause accounts for 0.04% of breast tumors. This condition is more common in young women, usually presents as a palpable mass, and has a poor prognosis, with five-year survival rates ranging from 8 to 50 percent.

Cardiac angiosarcoma

This is the most common malignant primary tumor in the heart, accounting for 10–25% of all cardiac tumors. Common symptoms include difficulty breathing, chest pain, hypotension, etc.

Hepatic angiosarcoma

This is the third most common type of liver cancer, but it accounts for only 0.1–2.0% of all liver cancers. Even with surgical removal, patient survival rates for this type of tumor are extremely low, with most patients dying within six months or a year.

Symptoms and diagnosis of angiosarcoma

Cutaneous angiosarcoma may initially appear as bruises or purple-red papules, sometimes similar in appearance to benign tumors, which can delay diagnosis. Symptoms of cardiac angiosarcoma include transient dyspnea, chest pain and other non-specific symptoms, which can lead to incorrect diagnosis.

In terms of diagnosis, angiosarcoma is usually initially examined through imaging, such as MRI and CT, and then requires biopsy for confirmation. The key marker for diagnosis is CD31, which has high sensitivity and specificity in diagnosis.

The rarity of this disease prevents many doctors from making a timely initial diagnosis, causing the patient's condition to worsen.

In conclusion, recognition and early diagnosis of angiosarcomas are crucial because of their high metastatic and lethal potential. When unexplained purple-red spots appear on the body, you should seek medical examination in time. Is there a more dangerous disease lurking?

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