Cancer is a complex disease where abnormal cells grow uncontrollably and can spread to other parts of the body. Among the many forms of cancer, angiosarcoma stands out as a particularly rare and aggressive type. This malignancy originates from the endothelial cells that line the inner surface of blood and lymph vessels throughout the body.
Understanding Angiosarcoma
Angiosarcoma is a very uncommon soft tissue sarcoma, accounting for less than 1% of all sarcomas. It arises from endothelial cells lining blood or lymphatic vessels. While angiosarcoma can develop almost anywhere in the body, it most frequently occurs on the skin, particularly on the head, neck, and scalp in elderly white men.
It can also manifest in other locations, including the breast, liver, spleen, bones, and deeper soft tissues. Factors associated with increased risk include:
Previous radiation therapy
Chronic lymphedema (swelling due to lymphatic system issues)
Exposure to certain chemicals like arsenic or vinyl chloride
Some genetic conditions
Recognizing Signs and Symptoms
The signs and symptoms of angiosarcoma can vary considerably depending on where the tumor develops. On the skin, it may initially appear as a bruise-like lesion or a reddish-purple patch that does not fade and tends to grow larger. These skin lesions can also be raised nodules that may bleed easily if scratched or bumped.
When angiosarcoma affects internal organs, symptoms are often less specific, including pain in the affected area, swelling, fatigue, malaise, or unexplained weight loss. For example, liver angiosarcoma might cause jaundice and persistent pain in the upper left abdomen, while cardiac angiosarcoma can lead to shortness of breath. Seek medical attention for any persistent or unusual changes, especially those that are new or worsening.
Diagnosing Angiosarcoma
Diagnosing angiosarcoma begins with a physical examination to identify lumps or unusual skin changes. Given the tumor’s varied presentation, imaging techniques are often employed to help locate the tumor and assess its extent. Common imaging tests include Magnetic Resonance Imaging (MRI), Computed Tomography (CT) scans, and Positron Emission Tomography (PET) scans.
MRI is particularly useful for detailed soft tissue characterization and evaluating the tumor’s relationship with surrounding structures. CT scans provide information on the tumor’s size and can help detect any spread to other parts of the body. PET scans identify areas of high metabolic activity, indicating tumor presence and aiding staging. A definitive diagnosis of angiosarcoma, however, requires a biopsy, where a tissue sample is removed for pathological examination. Pathologists examine the cells for characteristics consistent with angiosarcoma, often using immunohistochemical staining to confirm the vascular origin of the tumor cells.
Treatment Approaches
Treatment for angiosarcoma is complex due to its aggressive nature and potential for widespread growth. Surgical removal of the tumor is the primary treatment method when feasible. The goal is to remove as much cancerous tissue as possible, ideally achieving clear margins.
Surgery is often combined with other therapies to improve outcomes and reduce recurrence risk. Radiation therapy, which uses high-energy rays to destroy cancer cells, may be administered before or after surgery to eliminate remaining cancer cells or shrink the tumor. Chemotherapy, using drugs to kill cancer cells, is frequently used for angiosarcomas that have spread or when surgery is not an option. Paclitaxel has shown promise in treating advanced angiosarcoma. Treatment choice depends on factors including tumor location, size, and spread.
Outlook and Follow-Up
The outlook for individuals with angiosarcoma varies significantly due to its aggressive nature. Factors like tumor location, stage at diagnosis, and treatment response all play a role in prognosis. For instance, cutaneous angiosarcomas, particularly those on the head and neck, tend to have a poor prognosis, with reported 5-year survival rates ranging from 11% to 53%.
Given the high potential for recurrence and metastasis (up to 70% of patients), ongoing monitoring and follow-up care are important after initial treatment. This involves regular imaging scans, such as chest X-rays for lung metastases, and routine physical examinations. Supportive care also manages symptoms and improves quality of life throughout treatment and recovery.