Angiosarcoma

Angiosarcoma is a rare cancer of blood vessels that metastasizes rapidly and is very difficult to treat.

Symptoms and History

Angiosarcoma is seen most commonly on the face and scalp of older patients. It often looks like a red-blue bruise that does not heal and seems to slowly expand. It has no symptoms other than the surface level discoloration and therefore is often ignored until it has gotten to be quite large. The examining physician will notice this “bruise-like” area and will question about previous trauma and length of time that it has been present. In children, it may affect internal organs as well, like the liver and heart.

Diagnosis

Clinical exam. Depending on the region involved, patients can present with various symptoms. Ranging from skin discoloration or a soft-tissue mass when the skin is involved to large abdominal distension, pain and symptoms of liver failure when the liver is affected by angiosarcoma.

Imaging. Multiple areas body imaging is usually performed to see if the tumor has spread into underlying structures. This usually includes MRI (Magnetic Resonance Imaging) of the affected area, CT (Computer Tomography) of lungs, evaluation of bones, etc.

Laboratory. Additionally, there will likely be a request for the patient to undergo a small biopsy procedure.  The piece of the tumor will be sent to the laboratory for microscopic examination by a pathologist, and at this point, a final diagnosis is made.  Some gene mutations have been found in angiosarcoma, including mutations in the genes PTPRB and PLCG1, as well as the UP160–SLC43A3 fusion gene.

Rare tumors registry.

The North American Pediatric Angiosarcoma Registry was established to optimize the diagnosis and treatment of vascular cancers in children.

Treatment

The treatment depends upon how invasive the tumor is. The management of a child with angiosarcoma includes a multidisciplinary team with a pediatric oncologist specialized in rare solid tumors, pediatric surgical oncology, radiologist, pathologist, etc. At a minimum, it is likely that the patient will be referred to a surgeon to have it completely removed if possible.  After the surgical resection, additional treatments such as radiation therapy or chemotherapy will be recommended depending upon the location and the spread of the tumor.