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About Childhood Angiosarcoma

  • Angiosarcoma is a type of cancer that begins in the cells that line the blood vessels or lymph vessels. It is a soft-tissue sarcoma, a solid tumor that forms in tissues that connect, support, or surround organs and other body tissue.

    • Angiosarcoma is very rare. It occurs in only about two people per million. It is more common in adults than in children, and it is treated differently in each group.
    • This cancer can appear anywhere in the body. Common sites in adults are the head and neck region. In children, angiosarcoma can appear on the skin or in the deep tissues or internal organs. The tumor can spread to the lungs, liver, and bones.
    • Angiosarcoma's cause is currently unknown, although specialists have found genetic mutations in adult patients. Scientists are now searching for therapies that target these mutations.
    • In adults, angiosarcoma can occur after exposure to chemicals or radiation or because of ulcers or lymphedema.
    • Rarely, angiosarcomas in children form because of vascular anomalies and hemangiomas of the liver.
    • 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 rapid evaluation and treatment.
    • Angiosarcoma is malignant (cancerous). It is one of the most challenging types of vascular tumors to treat.

    At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, we have a specialized program to treat children with this rare cancer. We partner with the Vascular Anomalies Center at Boston Children's Hospital to evaluate and treat pediatric angiosarcoma. Our collaborative team includes one of the few pathologists with specialized expertise in pediatric vascular tumors. With this deep expertise, we're able to provide your child with the best possible care.

    How We Diagnose and Classify Childhood Angiosarcoma

    An accurate diagnosis is critical to forming the best possible treatment plan for angiosarcoma. If your child has a skin lesion that won't go away, gets larger, and is present after the age of 6 months, a vascular specialist should investigate it. Any liver lesion that enlarges during "hemangioma" therapy also should be evaluated by an expert.

    We develop an accurate and complete diagnosis through advanced imaging studies, biopsies, and bone scans. There may be other diagnostic tests that your doctor will discuss with you, depending on your child's situation.

    After completing all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.

    Doctors sometimes diagnose angiosarcoma as a hemangioma or another benign tumor. If you suspect your child may have received an incorrect diagnosis, it is worth seeking a second opinion. We understand how to differentiate between angiosarcoma and other vascular tumors.

    As a type of cancer, doctors classify angiosarcoma according to the following stages:

    • Stage I: The tumor is relatively small and contained within the area where it started.
    • Stage II: The angiosarcoma has not started to spread into surrounding tissue, but it is larger than in stage 1. (Both stage 1 and stage 2 are considered low-grade.)
    • Stage III: The angiosarcoma tumor is larger and has started to spread into surrounding tissues.
    • Stage IV: The angiosarcoma has spread from where it started to other organs or sites in the body. It is metastatic cancer. (Stage 3 and 4 are high-grade tumors.)

    How We Treat Childhood Angiosarcoma

    Your child's medical team will determine an individualized treatment plan based on your child's health and the tumor's type, location, and size.

    Angiosarcoma treatment options include:

    • Surgery: Surgeons with specialized expertise will remove as much of the tumor as possible. If the tumor is localized (hasn't spread beyond the original site), it may be possible to remove the entire tumor through surgery without further treatment. In most cases, however, it's not possible to remove the whole tumor, either because it has spread or because it significantly impacts a major organ such as the liver. We often use chemotherapy and radiation therapy in combination with surgery.
    • Chemotherapy: We may use this drug treatment that interferes with the cancer cells' ability to grow or reproduce. Different chemotherapy drugs work in different ways. Patients can receive chemotherapy by mouth, injection into the muscle or directly into the vein (IV), or direct injection into the tumor vasculature.
    • Radiation therapy: This treatment uses high-energy rays (radiation) from a specialized machine to damage or destroy cancer cells and shrink tumors.
    • Biologic agents: For high-grade angiosarcoma, treatment may also include biologic agents, usually angiogenesis inhibitors (medications that inhibit new blood vessels). Sometimes, these medications can shrink the tumor further.
    • Clinical trials: Children with relapsed angiosarcoma (the tumor returns) or refractory angiosarcoma (the tumor doesn't entirely go away despite treatment) may be eligible for clinical trials that test new treatment options.

    Children treated for angiosarcoma require ongoing, specialized cancer survivorship care. Patients visit a survivorship clinic annually to manage disease complications, screen for early recurrence of cancer, and manage late effects of treatment. Our pediatric cancer survivorship programs also include patient and family education, psychosocial assessment, and genetic counseling.

    Research and Clinical Trials for Childhood Angiosarcoma

    Both Dana-Farber Cancer Institute and Boston Children's Hospital are among the top pediatric research centers in the world for pediatric tumors and vascular anomalies. Much of our current angiosarcoma research focuses on understanding the genetic underpinnings of angiosarcoma better.

    The physician-scientists in our programs provide free on-site genomic sequencing of all angiosarcoma tumors (using samples of tissues obtained during a biopsy). Genomic sequencing can help provide insights into complex diseases by finding common genetic variants (mutations) shared between the individuals with the disease. Once researchers discover the specific mutations, it may be possible to develop drugs that can counteract those mutations. Ultimately, someday we may be able to treat angiosarcoma with precision medicine instead of surgery.

    Currently, researchers are performing genomic sequencing to increase scientific knowledge. However, if an individual's test reveals information that could be of clinical benefit, they will share those results with your referring physician for discussion with you and your child.

    For many children with rare or hard-to-treat conditions such as angiosarcoma, clinical trials provide new, promising options. We have several early-phase (phase 1 or 2) clinical trials that may be an option for children with relapsed or refractory solid tumors, including angiosarcoma.

    Long-term Outcomes 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, we hope the prognosis will improve.

    Childhood Angiosarcoma Treatment Team

    The Dana-Farber/Boston Children's solid tumor treatment team includes specialists who treat children with rare cancers such as angiosarcoma. See a complete list of the specialists in our Childhood Solid Tumor Center.