Introduction
Soft tissue sarcomas are a rare and aggressive form of cutaneous angiosarcoma (CAS) with a dismal prognosis and inadequate therapy choices. Skinny angiosarcoma frequently develops in the head and neck. However, clinical appearance varies. Angiosarcoma is an uncommon kind of cancer that develops in the lining of the lymphatic and blood arteries.
Any location on the body might develop angiosarcoma. However, it often shows up as skin changes in the neck and head. Rarely, it might appear on a different body area, including the breast. Or, it could develop in deeper tissues like the liver and the heart. In places that have already received radiation therapy, angiosarcoma can develop.
The most popular treatment, surgical excision with adjuvant radiation, has a high recurrence rate and can leave patients with severe deformity. Alternatives to focused therapy, like chemotherapy, have been less effective.
What Is Cutaneous Angiosarcoma?
One of the most malignant skin tumors, primary cutaneous angiosarcoma, has an extremely bad prognosis. The basic type of cutaneous angiosarcoma is a vaguely defined, edematous lesion that resembles a bruise and has, in its early stages, a mainly indolent clinical appearance.
Wilson-Jones angiosarcoma affects older people's faces or scalps, making up around 50 percent of all primary cutaneous angiosarcomas. Two other typical types of angiosarcoma include post-radiation angiosarcoma, which develops in areas of irradiated skin, typically in the pectoral region of women with a history of breast cancer treated with radiation therapy, and Stewart-Treves syndrome, which manifests in areas of long-standing lymphedema and is particularly frequent in women who have undergone radical mastectomy.
What Causes Cutaneous Angiosarcoma?
Angiosarcoma develops when the normal cells lining the blood arteries turn aberrant. In contrast to normal cells, aberrant cells do not die; instead, they keep producing other abnormal cells, which grow to produce masses or tumors. The afflicted blood vessels continue producing abnormal cells, occasionally spreading to other body parts.
According to researchers, the genetic coding of the blood arteries or lymph vessels is thought to alter when these tumors first appear. They have also discovered several risk variables that might raise the risk of angiosarcoma.
These risk elements include:
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Radiation Treatment - Especially if an individual had radiation treatment for breast cancer. According to researchers, Angiosarcoma, which can appear several years after radiation therapy, is thought to be related to radiation therapy.
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Exposure to pollutants, such as thorium dioxide, polyvinyl chloride, and arsenic.
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Chronic Lymphedema - The Stewart-Treves syndrome, a kind of chronic lymphedema, is associated with around 5 percent of all angiosarcomas.
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Familial Syndromes - People with illnesses brought on by genetic abnormalities are afflicted by around 3 percent of all angiosarcomas.
Who Is Affected by Cutaneous Angiosarcoma?
Even though angiosarcoma can strike anybody at any age, it is more common in adults 60 and older, and primary angiosarcoma strikes more men than women (AMAB) who were designated male at birth.
What Are the Symptoms Associated With Cutaneous Angiosarcoma?
The signs and symptoms of angiosarcoma might change depending on where the tumor develops.
A Skin-Related Angiosarcoma
Angiosarcoma often develops in the skin of the head and neck. On the scalp, it commonly occurs. The following are signs of this kind of angiosarcoma:
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A bruise-like mole or lump on the skin.
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A lesion that grows over time and mimics a bruise.
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A wound that might bleed if touched or jarred.
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Skin swelling surrounding the lesion.
Organ-Specific Angiosarcoma
When angiosarcoma affects organs like the liver or heart, it typically causes pain. Any further symptoms will depend on where the angiosarcoma is located.
What Are the Risk Factors Associated With Cutenous Angiosarcoma?
Angiosarcoma risk factors may include the following:
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Radiation treatment - Angiosarcoma risk may rise due to radiation therapy for cancer or other disorders. Radiation treatment occasionally causes the unusual adverse effect of angiosarcoma.
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Swelling is brought on by a damaged lymphatic vessel. Lymphedema is swelling brought on by an accumulation of lymph fluid. When the lymphatic system becomes clogged or injured, it occurs.
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The removal of lymph nodes during surgery might result in lymphedema. When treating cancer surgically, this is frequently done. Infections and other illnesses can cause lymphedema as well.
How Cutenous Angiosarcoma Can Be Diagnosed?
Due to its silent nature or lack of distinctive symptoms, angiosarcoma can be challenging to diagnose. MRI, CT, or ultrasound scans are frequently used for the first diagnosis. However, it can be challenging to determine if a lump is an angiosarcoma or another tumor such as melanoma or carcinoma. This implies that although imaging is frequently used for first diagnosis, a biopsy and additional histological and immunohistochemical analyses are required for definitive diagnosis.
CD31 marker is widely thought of as the gold standard for the diagnosis of angiosarcoma because of its great sensitivity and specificity. Other often used markers include CD34, vascular endothelial growth factor, and von Willebrand factor. Angiosarcomas and melanomas can be differentiated by looking for markers such as S100 and HMB-45 absent in melanomas.
How Do Healthcare Providers Treat Cutaneous Angiosarcomas?
The most frequent therapy for angiosarcoma is surgery to remove the tumor. Before or after surgery, medical professionals could advise radiation treatment or chemotherapy. Adjuvant treatment is this. Each therapy will differ based on circumstances and elements like age, geography, and general health. The healthcare professional will explain each therapy and any possible adverse effects.
Conclusion
Angiosarcoma is a rare and serious illness that starts in endothelial cells that line the walls of blood vessels or lymphatic systems. Since they are vascular lining-based, they can develop anywhere and in anybody, but older people are more likely to be impacted. The skin is the most usually affected location, with around 60 percent of cases being cutaneous. Particularly, 50 percent of angiosarcoma instances involve the scalp.
Surgery, chemotherapy, and radiation therapy are all types of treatment; often, all three are used together. These tumors can quickly spread to other organs, especially the liver and lungs since they start in the cells lining blood or lymphatic arteries. This makes them particularly deadly, and survival typically depends on an early diagnosis.
