Essential Information about Angiosarcomas

Malignant tumors originating from connective and supportive tissues, known as sarcomas, encompass various types. These tumors grow rapidly, with approximately 70 subtypes identified. Angiosarcomas, which arise from the inner layer of blood and lymphatic vessels, represent a specific form of sarcoma. The outcome of the disease primarily depends on the diversity of histological patterns, which are also taken into account by international medical guidelines.

Causes of Angiosarcoma Development

The exact causes of angiosarcoma development are often unknown. However, certain factors may contribute to the appearance of the disease. For example, when the tumor develops in the limbs, it is often associated with lymphatic circulation disorders caused by bacterial or parasitic infections.

The historical background of radiation treatments can also play an important role in the development of tumors. Angiosarcoma is often observed after radiation treatments used for breast cancer. The degree and location of radiation, as well as individual genetic predispositions, all influence the likelihood of tumor development. Older radiation treatment techniques, such as the box technique, have particularly adverse effects on the skin, and the appearance of tumors can occur even decades later.

Workplace exposures can also contribute to the development of the disease. For instance, contact with PVC, which was prevalent in plastic manufacturing, previously resulted in high rates of angiosarcoma among workers. Additionally, X-ray contrast agents like thorotrast have also increased the likelihood of tumor development. Exposure to arsenic-containing materials and long-term use of anabolic steroids have also been linked to the appearance of angiosarcomas.

Clinical Symptoms of Angiosarcoma

The symptoms of angiosarcomas can vary depending on whether they develop on the skin or inside the body. Tumors that develop beneath the skin typically present as small swellings that may be reddish or purplish in color and can cause bleeding. If the wound does not heal or the tumor continues to grow, suspicion of a malignant lesion may arise.

Angiosarcomas located inside the body are most often detected due to pressure on surrounding tissues or discovered during imaging studies. More than half of tumors originating from internal organs exceed 5 centimeters in size at the time of diagnosis. Larger tumors can rupture, leading to internal bleeding.

Diagnosis and Investigation of the Disease

Examining the skin surface is the first step in establishing a diagnosis, but a dermatological examination is necessary in the presence of suspicious symptoms. For asymptomatic individuals, ultrasound examination may help determine the presence of angiosarcoma. Further imaging techniques, such as CT or MRI, are required to accurately define the tumor’s vascular supply and location. A PET-CT scan may also be useful for detecting distant metastases.

Histological evaluation is also essential for confirming the diagnosis. Various histological forms of angiosarcomas exist, but these do not necessarily influence treatment options. The aggressiveness of the tumor is determined by the diversity of the cells and their degree of proliferation, contributing to the prognosis of the disease.

Treatment Options and Management

The treatment of angiosarcomas is complex, and due to the rarity of the tumors, there is no unified international protocol. The treatment of sarcomas, and thus angiosarcomas, is recommended at centers with extensive experience.

Surgical intervention is the most common primary treatment, aiming to ensure that no cancerous cells remain at the tumor’s margins. Due to the risk of local recurrence, continuous monitoring of patients is necessary. Regarding tumor management, if the tumor is supplied by only a few large vessels, their embolization may reduce the tumor size.

Radiation therapy can also be applied to prevent local recurrence. Research has shown that postoperative radiation therapy can significantly increase patient survival. In the case of tumors located in internal organs, radiation therapy is only applicable to a limited extent.

In cases of chemotherapy treatment, if no cancerous cells were found during tumor removal, adjuvant chemotherapy is generally not necessary. For metastatic angiosarcoma, doxorubicin and its derivatives are the most effective, but other drugs, such as paclitaxel and gemcitabine, may also be used, considering the patient’s condition.

Prognosis and Outcome

The prognosis of the disease significantly depends on the tumor’s location. For angiosarcomas appearing on the surface of the skin, the average survival exceeds 5 years, while for tumors originating from internal organs, survival often is limited to just six months. Considering the type of tumor, its location, and the effectiveness of treatment, the best options for recovery should be provided to the patients.