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:
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:
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:
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.
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
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
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.
Phone: 617-632-5508Online form: Request an appointment