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The mystery of vascular cancer: Do you know how it grows silently in the body?
Angiosarcoma is a rare and aggressive cancer that originates in the endothelial cells that line the lining of blood or lymphatic vessels. This cancer can appear anywhere in the body and at any age, but it is most common in older people and the skin is the most affected area, with about 60% of cases being cutaneous. The scalp in particular accounts for about 50% of all vascular cancer cases, however this is still not enough to reach 0.1% of the total number of head and neck cancers.
Because vascular cancer is an umbrella term that covers tumors of many origins and locations, many symptoms may occur, ranging from no symptoms at all to non-specific symptoms such as skin lesions, ulcers, shortness of breath, and abdominal pain.
Simultaneous damage to multiple organs is common at diagnosis, making it difficult to determine the tumor's origin and treatment options. The cause of vascular cancer is still unknown, but several risk factors are known, such as chronic lymphedema, radiation therapy, and various chemicals such as arsenic and vinyl chloride. Vascular cancer is also sometimes associated with the long-term presence of foreign bodies and occasionally with breast implants.
Early diagnosis is crucial to its survival rate, but despite treatment, the prognosis remains poor, with a five-year survival rate of only 30 to 38 percent.
Detection of vascular cancer can be done with MRI, CT and ultrasound scans, but is often difficult to distinguish from other cancers and requires confirmation by biopsy and immunohistochemistry analysis. Treatment options include surgery, chemotherapy and radiation, often a combination of all three. Because these cancers originate from cells in blood vessels or lymphatic vessels, they can easily metastasize to distant sites, especially the liver and lungs, making them particularly deadly.
Classification of vascular cancer
Vascular cancers are classified according to their origin and underlying risk factors. This is not a comprehensive list and case reports often go into further detail.
Primary cutaneous vascular carcinoma
This type of vascular cancer is usually located in the skin (often the head or neck, especially the scalp) without underlying risk factors such as chronic lymphedema or radiation therapy.
Vascular cancer associated with lymphedema
Also known as Stewart-Treves syndrome, this is a lymphovascular cancer caused by chronic lymphedema. It usually occurs in the breast, especially in patients who have had breast lymph nodes removed, but can occur elsewhere.
Substantial organ vascular carcinoma
Vascular cancers occurring in solid organs such as the liver, breast, or heart account for approximately 40% of vascular cancers.
Primary breast vascular carcinoma
Refers to vascular cancer of the breast that occurs in the absence of an underlying cause such as radiation therapy or lymphedema. This condition is relatively rare, accounting for only 0.04% of breast tumors and 8% of breast soft tissue sarcomas. It is quite aggressive and is usually found in younger women, often showing up as a palpable lump. The prognosis is poor, with five-year survival rates ranging from 8% to 50%.
Secondary breast vascular carcinoma
These are vascular cancers of the breast caused by underlying factors such as lymphedema or radiation, often occurring after radiation therapy for breast cancer. Regarding the relationship between radiation therapy and vascular cancer, some experts have raised objections, believing that the occurrence of vascular cancer is due to the simultaneous occurrence of lymphedema. The prognosis for secondary vascular breast cancer is poor, and the five-year survival rate for radiation-related vascular cancer is only 10% to 54%.
Primary cardiovascular cancer
Primary cardiovascular cancer refers to cancer of the blood vessels that arise in the heart. Although very rare, it is the most common malignant heart tumor, with approximately 10% to 25% of cases being vascular cancer. Symptoms include shortness of breath, chest pain, low blood pressure, and more. Because symptoms are nonspecific and the disease is rare, doctors often miss it and delay initial diagnosis.
In most cases, even with surgery and treatment, the prognosis remains poor, with the average survival time ranging from three months to four years after diagnosis.
Prevention and Diagnosis
The underlying cause of vascular cancer is unknown, but known risk factors include lymphedema, radiation therapy, and certain chemicals. Due to the non-specific symptoms and insidious nature of vascular cancer, timely and correct diagnosis is crucial. Imaging studies are often difficult to diagnose, so follow-up tissue biopsies are necessary to confirm the condition.
Epidemiological data for this cancer show that vascular cancer is diagnosed annually in the United States at approximately one case per million people, primarily affecting older adults, with no significant gender differences. However, the incidence of vascular cancer of the liver is higher in men, with a ratio of 3 to 4:1. The incidence of this disease has increased in the United States in recent years.
Are you ready to face this mysterious and deadly disease?
