If your child has been diagnosed with a tufted angioma, you probably have many questions:
- What is it?
- Will it go away?
- How is it treated?
- What does it mean for my child?
We’ve tried to provide some answers to these questions in these pages, and when you meet with our experts, we can further explain your child’s condition and treatment options.
What does a tufted angioma lesion look like?
The lesions usually have the following characteristics:
- usually a deep, reddish-purple
- firm and warm to the touch
- There may be tiny purple or red spots and a bruise-like discoloration near the lesion.
Where does the lesion appear?
The lesion can appear anywhere on your child’s body. It’s often found on the head and neck, but can also appear on the stomach, back, arms and legs.
Is my child’s lesion painful?
It can be, but many cases are painless unless the lesion is bumped. As the tumor gets bigger and more aggressive, your child’s lesion can swell and may be very painful. This may also occur with infection, trauma to the lesion or after a platelet transfusion.
Does tufted angioma always lead to Kasabach-Merritt phenomenon?
Not always. We cannot currently predict which tufted angioma lesions will trap platelets and have the associated bleeding risk.
There’s also a different kind of tufted angioma that we call acquired tufted angioma. This lesion appears in childhood as a small, hard, dull red or purple patch on the skin. This type of tufted angioma appears less likely to trap and destroy platelets, though it can still cause pain.
How common is it?
It’s very rare, representing less than 1% of birthmarks. The incidence is unknown, but is likely less than 1 in 100,000.
What causes tufted angioma?
Tufted angioma is not inherited — researchers are studying the cause of these rare tumors. Abnormal blood vessel formation and growth typically occurs in infancy, although it can happen prenatally and also in adults after trauma.
What are the symptoms of tufted angioma?
Often, tufted angioma can be misdiagnosed by your child’s primary physician, simply because the condition is so rare. Here are some signs that you can look for; if your child has one or more of these symptoms, we recommend getting a second opinion from a vascular anomalies specialist.
- a lesion on the skin that’s getting larger than expected
- a lesion that swells and becomes painful
- pain with movement or decreased range of motion around the area of the lesion
Tufted angioma may also show up with different symptoms based on the location of the lesion.
What is the long-term outlook for my child?
Tufted angioma is such a rare tumor that it is difficult to determine the long-term outlook for your child. Every child is unique and your care team will work with you to develop a treatment plan that works for your family. We will openly share our experience about outlook and prognosis with you.
Because tufted angioma doesn’t always spread and begin to trap platelets, often your child’s doctor may recommend observation as a first-line therapy.
If the tumor does progress, it’s important to get prompt medical attention to allow early discussions about treatment. This enables your doctor to attempt to shrink the tumor before it affects adjacent muscle or tissue.
The prognosis for your child greatly depends on:
- the extent of the disease
- the size and location of the tumor
- the tumor's initial response to therapy
- new developments in treatment
Will my child’s tumor come back after treatment?
There is some evidence that these tumors may recur, though it is currently unclear how common this is. If your child’s tumor does recur, we're usually able to treat it again, reduce its size and stabilize your child's platelet count.
What do we do after treatment is over?
Your child should continue with regular, thorough clinic visits at least every year. The purpose of these visits is to monitor for recurrence and rare late effects from therapy. A typical follow-up visit may include some or all of the following:
- a physical exam
- blood tests
- imaging or scans
Q: Is tufted angioma a birthmark?
A: No. tufted angioma is a vascular tumor that causes a discolored lesion or
birthmark on your child’s skin.
Q: Is tufted angioma a hemangioma?
A: No. Hemangiomas have a different appearance, grow predictably and shrink and fade on their own. They also look different when biopsied. Tufted angioma is not associated with infantile hemangiomas, although it is sometimes misdiagnosed as a hemangioma.
Q: Does my child have cancer?
A: No. We use the term “tumor” to mean abnormal growth. While we refer to tufted angioma as a tumor, it’s a benign tumor, which means it doesn't metastasize and spread to other locations in your child’s body.
Q: So it’s not cancer. Why is it a problem?
A: Tufted angioma can be a problem because the tumor can affect adjacent muscle/tissue.This can result in three different kinds of problems:
- pain/difficulty moving — If the tumor spreads into adjacent muscle tissue, your child may have difficulty moving the affected area (such as an arm or a leg). There may also be pain involved.
- damage to an internal organ — If the tumor spreads too close to an internal organ, it can hurt that organ.
- bleeding — As the tumor grows, it captures more and more platelets, putting your child at risk for bleeding. This is referred to as Kasabach-Merritt phenomenon.
Q: Is tufted angioma treatable?
A: Yes, although treatment is not always necessary. If your child’s doctor decides that the tumor is growing or needs therapy, tufted angioma can be treated in several ways. Our doctors use different medications to stop the growth of the tumor, improve your child’s platelet count and help prevent any complications.
Q: What kinds of medications are used?
A: Our doctors will often recommend steroids first and then move on to chemotherapy or interferon therapy if steroids are not effective.
Q: What makes Boston Children’s different?
A: We have more experience evaluating, diagnosing and treating children with tufted angioma than any other hospital in the world. We assist doctors across the globe with diagnosis and treatment for vascular anomalies like tufted angioma, evaluating about 1,000 cases of vascular anomalies annually.
Q: My child has a low platelet count and a birthmark that was diagnosed as a hemangioma. Should I get a second opinion?
A: Yes. A low platelet count is never caused by common infantile hemangioma. Low platelets combined with a skin lesion should lead to evaluation for tufted angioma or other benign vascular tumors like Kaposiform hemangioendothelioma or cutaneovisceral angiomatosis with thrombocytopenia (CAT) — also called multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT).
If your child’s platelet count is low and he has a vascular tumor or birthmark, you should definitely consult a vascular anomalies center. Keep in mind, however, that tufted angioma is very rare and having these two symptoms does not necessarily mean that your child has a vascular tumor. There are other reasons your child may have a low platelet count and our hematologist-oncologist can clarify the cause in your child.
Q: My child has a low platelet count and our doctor recommended a transfusion. Now the lesion is bigger than ever and more painful. What happened?
A: What happens in a platelet transfusion is that the new platelets are quickly attracted to the tumor and trapped. Your child’s lesion will quickly grow and become extremely painful. Over time, the lesion will shrink back to its normal size over time, but your child’s platelet count will not improve from the transfusion.
We only recommend a platelet transfusion if your child is bleeding badly — not simply because the platelet count is low.
It’s important to treat the tumor, not the platelet count. We focus on shrinking the tumor, understanding that the platelet count will normalize as your child’s tumor becomes less aggressive. Your child’s platelet count will go up as the tumor shrinks (because it’s not trapping as many platelets).
Questions to ask your child’s doctor
After your child is diagnosed with a tufted angioma, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.
Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.
Some of the questions you may want to ask include:
- How will my child’s condition be treated?
- Will my child need to stay in the hospital?
- What side effects or complications might my child experience due to treatment?
- What kind of follow-up care should my child receive? How often will she need to come in?
- What is the likelihood of cure?
- How much experience does this center and my child’s doctor have with treating tufted angiomas?