Angiosarcoma is a type of cancer that begins in the cells
that line the blood vessels or lymph vessels. It is a type of soft-tissue
sarcoma, which are solid
tumors that begin in tissues that connect, support, or surround organs and
other body tissue.
Because angiosarcoma is so rare, very few doctors have experience
diagnosing and treating it. The Vascular
Anomalies Center at Boston Children's Hospital, in partnership with
the Dana-Farber/Boston Children's Solid
Tumor Center, has evaluated
more children with angiosarcoma and other rare vascular tumors than any other
hospital in the world.
Our physicians – representing 16 medical and surgical specialties,
including radiologists and pathologists who specialize in diagnosing vascular
anomalies – draw on those experiences to continually refine the therapies and
achieve better long-term outcomes for children with angiosarcoma.
Our team takes an interdisciplinary approach to care with every child
we see. On your child’s first visit, your child likely will be evaluated by a
whole treatment team at the same time. From there, the team works with you to
develop and carry out a comprehensive and coordinated care plan that matches
your child's specific needs.
We also can provide care to angiosarcoma patients from afar, seeing
the child in person on an infrequent basis but coordinating closely with
providers closer to home.
Angiosarcoma is a type of vascular tumor. The World Health
Organization (WHO) classifies vascular tumors into three types: benign,
intermediate (either locally aggressive or rarely metasticizing), and
malignant. Angiosarcoma fits into that third category – malignant (cancerous). It
is one of the most difficult types of vascular tumor to treat.
As a type of cancer, angiosarcoma is also classified according
to the following stages:
- Stage 1: the tumor is relatively small and contained within
the area where it started.
- Stage 2: 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 3: the angiosarcoma tumor is larger and has started to
spread into surrounding tissues.
- Stage 4: the angiosarcoma
has spread from where it started to other organs or sites in the body. This is
called metastatic cancer. (Stage 3 and 4 are high-grade tumors.)
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, including a complete blood count and blood
chemistry.
- Ultrasound (also called sonography) – used to view internal organs
as they function, and to assess blood flow through various vessels.
- Magnetic resonance imaging (MRI), which is a test that uses a
combination of large magnets, radiofrequencies, and a computer to
produce detailed images of organs and structures within the body.
- Bone scans or PET scan, which include taking pictures or x-rays of
the bone after a dye has been injected that is absorbed by
bone tissue. These are used to detect tumors and bone
abnormalities.
- Computerized
tomography scan (CT or CAT scan)—A CT scan shows detailed images of any
part of the body, including the bones, muscles and fat. When angiosarcoma
is suspected, a CT scan of the lungs is important to rule out pulmonary
disease or to monitor a known pulmonary disease.
- Biopsy, a sample taken of the primary tumor and/or metastatic lesions
to provide definitive information about tumor type. The biopsy sample can
be used for genomic testing, to determine the specific mutations within
the tumor
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. Then we will 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.
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:
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, in which case no
further treatment is required. In most cases, however, it’s not possible to
remove the entire tumor, either because it has spread or because it
significantly impacts a major organ such as the liver.
- Chemotherapy and radiation therapy are often
used in combination with surgery.
- Chemotherapy is drug treatment that
works by interfering with the cancer cells’ ability to grow or reproduce. Different
chemotherapy drugs work in different ways. Chemotherapy may be given by mouth,
by injection into the muscle or directly into the vein (IV), or by direct
injection into the tumor vasculature.
- Radiation therapy uses high-energy
rays (radiation) from a specialized machine to damage or kill cancer cells and
shrink tumors.
- Biologic
agents: 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. (See next section for more details.)
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. Our research programs include
laboratory scientists and clinical researchers.
Much of our
current angiosarcoma research is focused on trying to better understand the
genetic underpinnings of angiosarcoma. The physician scientists in our programs
provide free on-site genomic sequencing of all angiosarcoma tumors (using
samples of tissues obtained during biopsy). Genomic sequencing can help provide
insights into complex diseases by finding common genetic variants (mutations)
shared between the individuals with the disease. Once the specific mutations
are discovered, it may then be possible to develop drugs that can counteract
those mutations. Ultimately, someday we may be able to treat angiosarcoma
without needing surgery, instead using precision medicine.
Currently, the
genomic sequencing is being performed primarily to increase scientific
knowledge. However, if an individual’s test reveals information that could be
of clinical benefit, those results will be shared with your referring physician
for discussion with you and your child.
Angiosarcoma Clinical Trials
For many children with rare or
hard-to-treat conditions such as angiosarcoma, clinical trials – research studies evaluating new treatment
approaches – provide new options. We have a large portfolio of early
phase (phase 1 or 2) clinical trials that may be an option for children with
relapsed or refractory solid tumors, including angiosarcoma.
Participation in any clinical trial is completely voluntary. We will
take care to fully explain all elements of the treatment plan prior to the
start of the trial, and you may remove your child from the medical study at any
time.
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 that the prognosis will
improve.
Survivorship Care
After finishing treatment for angiosarcoma, children should visit a cancer
survivorship clinic every year to manage disease complications, screen for recurrence and manage late treatment side effects. A typical follow-up visit is likely to include a physical exam,
laboratory testing and imaging scans. The David B. Perini, Jr. Quality of Life Clinic at Dana-Farber provides care and advocacy for survivors of childhood cancer, conducting research on the long-term effects of cancer treatments, and offering education and support for
survivors of cancer.