Angiosarcoma | Symptoms & Causes
What are the symptoms of angiosarcoma?
Angiosarcoma can appear anywhere in the body. Common sites in adults are in the head and neck region. In children, angiosarcoma can arise on the skin, deep tissues, or internal organs. The tumor can spread to the lung, liver and bone.
The tumor behaves differently in each person. Usually it is very aggressive, but some tumors are low-grade (do not appear as aggressive under the microscope). All angiosarcomas need aggressive evaluation and treatment.
What causes angiosarcoma?
The cause of angiosarcoma is currently unknown, although genetic mutations recently have been identified in adult patients; scientists are now searching for therapies that target these mutations. In adults, angiosarcoma can arise after exposure to chemicals or radiation, or in long-standing ulcerations and lymphedema. Though very rare, angiosarcomas in children have been reported to arise in vascular anomalies and hemangiomas of the liver.
Angiosarcoma | Diagnosis & Treatments
How are angiosarcomas diagnosed?
The first step in treating your child is forming an accurate and complete diagnosis. Your child’s treatment team may order a number of different diagnostic tests for angiosarcoma, including:
- blood and urine tests
- ultrasound
- magnetic resonance imaging (MRI)
- bone scans or PET scan
- computerized tomography scan (CT or CAT scan)
- biopsy
There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. We will then meet with you and your family to discuss the results and outline the best possible treatment options.
Because angiosarcoma is often misdiagnosed as a hemangioma or other benign tumor, if you suspect your child may have received an incorrect diagnosis, it is worth seeking a second opinion. Our treatment team includes one of the few pathologists with specialized expertise in pediatric vascular tumors, including how to differentiate between angiosarcoma and other vascular tumors. An accurate diagnosis is key to forming the best possible treatment plan. Any skin lesions that do not go away, get larger and are noted after the age of 6 months should be investigated by a vascular specialist. Any liver lesion that enlarges during “hemangioma” therapy also should be evaluated by an expert.
What are the treatment options for angiosarcoma?
Your child's treatment team will determine a specific course of angiosarcoma treatment based on several factors, including your child's age, overall health and medical history and the type, location, and size of the angiosarcoma. Angiosarcoma treatment options include:
- surgery
- chemotherapy
- radiation therapy
For high-grade angiosarcoma, treatment may also include biologic agents, usually angiogenesis inhibitors — medications that inhibit the growth of new blood vessels. Sometimes, these medications can shrink the tumor further.
Children with relapsed angiosarcoma (the tumor returns) or refractory angiosarcoma (the tumor doesn’t fully go away despite treatment) may be eligible for clinical trials that test new treatment options.
What is the long-term outlook for children with angiosarcoma?
The prognosis for children with localized (stage 1) angiosarcoma that can be completely surgically removed is good. However, for children with advanced angiosarcoma, the prognosis remains poor. As physician scientists learn more about the genetic underpinnings of angiosarcoma, and seek to develop drugs that target specific mutations, the hope is the prognosis will improve.
