Tufted angioma

Having identified your child’s condition, we’re able to begin the process of treating her, so that we may ultimately return your child to good health. At Children’s Hospital Boston, we consider you and your child integral parts of the care team and not simply recipients of care. You and your care team will work together to customize a plan of care for your child.

Tufted angioma is a complex condition. If your doctor and vascular anomalies specialist decide that the tumor is growing or worsening, your child will likely be treated by some or all of the following specialists:

• hematologist-oncologist
• dermatologist
• plastic surgeon
• general surgeon
• interventional radiologist

Your care team (your child’s doctor and an experienced vascular anomalies specialist) will outline the benefits of all the treatment options listed below with you.

observation — If your child has a mild lesion with no pain and a normal platelet count, your doctor may want to simply observe the tumor for a period of time. This is often the case with tufted angioma.

steroids — If your doctor thinks treatment is necessary, she may recommend treating the tumor with steroids first. While they’re not effective in most cases, the treatment is easier (taken by mouth) than other options and well tolerated.

chemotherapy — If the steroids don’t work, your doctor may recommend chemotherapy to slow the growth of blood vessel cells. While the word “chemotherapy” can be scary, rest assured that the treatment for tufted angioma has few of the side effects of traditional chemotherapy (no hair loss and minimal or no nausea or effects on the immune system).

The first chemotherapy drug we recommend is vincristine. Vincristine slows down the growth of the tumor and can even stop growth or shrink tumors in some cases.

• We administer chemotherapy through an IV central line (also called a central venous catheter).
  • A surgeon will implant a small port-a-cath (you won’t see it, but you will be able to feel a bump) in your child’s chest, which allows the medicine to go directly into a vein.
  • It’s a minor surgery in which the surgeon makes a small incision and then inserts the port, linking it to a large vein in your child’s chest.
  • Once the course of treatment is completed, a surgeon will remove the port-a-cath.

• Every one or two weeks for about six months, your child will receive an IV dose of vincristine.
  • This is administered by experienced nurses and physicians either at Children’s or through our partnership with the Jimmy Fund Clinic next door at the Dana-Farber Cancer Institute. This therapy can also be coordinated with a clinic or hospital convenient for you.

Most children tolerate vincristine very well. Your care team will discuss the few, minor side effects your child may experience. Being able to anticipate these side effects can help you prepare for, and, in some cases, prevent these symptoms from occurring.

Interferon therapy — In cases where vincristine is not effective and your child is at least 12 months old, your doctor may recommend injections of interferon. Interferon is a drug that is used for a variety of disorders to limit new blood vessel formation and reduce blood supply to the tumor.

The regimen and length of therapy is variable and will be customized for your child by your doctor and care team. Injections can be given daily, three times a week or weekly and are usually continued until your child’s platelet count rises and the pain around the lesion subsides.

other treatments —  In very aggressive cases (or situations where your child’s tumor doesn’t respond to any of these therapies), your doctor has other options:

• clinical trials — As the home of the largest vascular anomalies center in the world, we often have access to new drugs involved in clinical trials. Search current and upcoming clinical trials at Children’s.
• embolization — This is a process where your doctor inserts a block into the main artery that’s feeding your child’s tumor. With the blood supply cut off, the tumor will often shrink. However, tufted angioma is most often fed by a number of arteries instead of one main artery; and if that’s the case, embolization is less successful and we usually don’t attempt it.
• surgery — Your doctor will only recommend surgery if your child’s tumor is very small (and we’re able to completely remove it) or extremely aggressive. Because of the location and size of these tumors, they are quite difficult to remove and the surgery can often cause more harm than good.
• radiation — This was historically used to try to control the growth of these tumors and is associated with significant short- and long-term side effects. We may consider using it in conjunction with one of the other treatments in very rare cases.

My child’s doctor recommended a platelet transfusion. Is that a good idea?

Very rarely. In general, we only recommend platelet transfusion if your child is bleeding badly.

Follow-up care

We want to maximize the safety and effectiveness of whatever therapy you and your child’s doctor choose. This involves preventing and treating infections and side effects of treatment to keep your child as comfortable as possible while we’re working to shrink the tumor.

Post-treatment, a schedule of follow-up care will be determined by your child's physician and other members of your care team to see how your child is responding to the therapy and check for recurrence of the tumor, a drop in platelet count or a worsening of the lesion.

We’ll also want to monitor for any problems because of the tissue involved as the tumor grows. Sometimes there can be scarring that could limit your child’s movement or be uncomfortable.

After we have succeeded in shrinking the tumor, there are often some residual marks on your child’s skin. Depending on where they are and how large they are, you may want to consult with one of our plastic surgeons to discuss corrective options.

Rehabilitation

Children with tufted angioma sometimes need multidisciplinary care. Each of these disciplines is available at Children’s with experts dedicated to the care of children with complex medical conditions. Specialists your doctor may recommend for your child include:

• physical therapists — Sometimes the tumor can affect surrounding tissue and muscles, making it hard or painful for your child to move an arm or a leg. A physical therapist can help ease the pain and increase your child’s range of movement, strength and flexibility.

• occupational therapists — Children with tufted angioma sometimes get behind on developing motor skills because of physical problems related to their tumors. An occupational therapist can get your child back on track.

• counselors — As your child gets older, he or she may be uncomfortable with the skin lesion, especially if it’s in a prominent location such as on the face or neck. One of our counselors can help your child deal with the psychological and social issues related to having a birthmark.

Coping and support

Hearing that your child has tufted angioma may be scary. We hope that this site reassures you with reliable information and begins to prepare you for the next steps. There are also a number of other resources to help you and your family through this difficult time.

Patient education: From the first office visit, our nurses will be on hand to walk you through your child’s treatment and help answer any questions you may have — What kinds of treatment are available? What symptoms might my child have? They will also reach out to you by phone, continuing the care and support you received while at Children’s.

Parent to parent: Want to talk with someone whose child has been treated for tufted angioma? We may be able to put you in touch with other families who can share their experience.

Faith-based support: If you are in need of spiritual support, we will help connect you with the Children’s chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.

Social work and mental health professionals: Our social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with issues such as coping with your child’s diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

Our Vascular Anomalies Center offers links to organizations that offer support and education for parents who have a child with a vascular anomaly. And on our For Patients and Families site, you can read all you need to know about:

• getting to Children’s
• accommodations
• navigating the hospital experience
• resources that are available for your family