Treatments for Epithelioid Hemangioendothelioma in Children

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What are the treatment options for my child?

EHE is a complex condition. Children with EHE often need care from doctors with many different backgrounds. Some specialists your doctor may recommend for your child include the following:

  • hematologist/oncologist
  • dermatologist
  • plastic surgeon
  • general surgeon
  • interventional radiologist
  • orthopedic surgeon
  • gastroenterologist/hepatologist
  • pulmonologist

Your care team will outline the benefits of all the EHE treatment options listed below:

Observation—If there are no symptoms or evidence of progression, your child’s doctor may want to simply observe the tumor for a period of time. Observation allows your child’s care team to determine how the tumor is behaving and if treatment should be considered. In rare cases, the tumor may shrink or even go away without treatment. If the team notes growth in the tumor, we will review options for further management with you.

Surgery—If your child’s tumor is small and/or in an easily accessible spot, your doctor may recommend an operation to remove it.

Medication—If your doctor thinks it’s not possible to remove the whole tumor surgically — or if there are multiple tumors in several locations—we may discuss medical therapy options to slow the growth of the tumor by interfering with abnormal cell growth.

The goal of medical therapy is to relieve symptoms and prevent further growth of the tumor. Medications may be used in combination and sometimes the first regimen isn’t successful and others may be recommended. While there is no current drug that specifically targets EHE cells, some tumors shrink and even become invisible with therapy.

Some of these medications target blood vessel formation (anti-angiogenic), while others are categorized as “chemotherapy,” meaning they may also be used for cancers. They need to be given and monitored by an experienced hematology/oncology team. We work closely with Dana-Farber/Boston Children’s Cancer and Blood Disorders Center to administer chemotherapy for EHE.

Individual medications on this list do not lead to all the side effects many people associate with chemotherapy. Your doctor will explain common and uncommon side effects for the particular medications relevant to your or your child’s case.

  • Sirolimus—This drug appears to target blood vessel growth. The VAC is rapidly gaining experience using sirolimus to treat patients with vascular anomalies, in part through recent clinical trials. We have used sirolimus for a small number of patients with EHE with some evidence of early benefit.
  • Tyrosine kinase inhibitors—These drugs were designed as targeted therapies for cancers, but have been used for many tumors. Some hospitals have used these drugs for short periods for EHE with short-term success and these drugs may be considered for your child. Examples include sorafenib, sunitinib and pazopanib.
  • Vincristine—This chemotherapy drug targets all dividing cells within the body and is therefore used to treat many cancers. It is used for aggressive benign vascular tumors like kaposiform hemangioendothelioma.
  • InterferonThe body produces this to combat infections or control inflammation. Interferon has been formulated into a medication that targets blood vessel growth.
  • Multi-agent chemotherapy—Aggressive EHE cases with rapid growth or spread to other tissues, or tumors that do not respond to the treatments listed above, may require more aggressive medical therapy. There are combinations of medications used successfully to treat other aggressive vascular tumors. Aggressive therapy is rarely needed in children and young adults with EHE.

Other treatments for epithelioid hemangioendothelioma

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

  • Embolization — This is a process in which a doctor blocks the main artery that’s feeding your child’s tumor. With the blood supply cut off, the tumor will often shrink. However, EHE is most often fed by a number of arteries instead of one main artery; and if that’s the case, embolization is much less likely to be successful.
  • Radiation — If we can’t remove your child’s tumor surgically and it doesn’t respond to chemotherapy or other medications, we may consider using radiation therapy in rare cases for solitary tumors or individual symptomatic tumors.

Supportive care

We want to maximize the safety and effectiveness of whatever therapy you and your child’s doctor decide upon. Supportive care involves preventing and treating infections and side effects of treatment to keep your child as comfortable as possible.

Follow-up care

Your child should have regular follow-up during and after treatment to check for recurrence of the tumor, any drops in platelet count or growth of the lesion. Your child’s health care team will give you a schedule of follow-up care to see how your child is responding to therapy, and to monitor for any late effects of therapy.

Other specialists that care for patients with EHE

Depending on your child’s symptoms and the progression of the disease, we may bring in the following specialists.

  • 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.
  • Occupational therapists—Children with EHE 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—Sometimes EHE shows up as a lesion on your child’s skin. As your child gets older, he or she may be uncomfortable with that 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. Counselors often serve important roles for siblings as well.

What is the long-term outlook for my child?

EHE is such a rare condition and behaves so differently from person to person that it’s difficult to determine the long-term outlook for your child.

The prognosis for your child greatly depends on:

  • the extent of the disease (how large the tumor is and whether it has spread)
  • the size and location of the tumor, which determine whether we can surgically remove it (tumors arising in the liver and those that are in multiple locations may be more aggressive)
  • the results of the biopsied tissue (areas of cell death and markers of cell replication may indicate more aggressive tumors)
  • response to therapy
  • the overall health of your child
  • your child's tolerance of specific medications, procedures, or therapies
  • new developments in treatment

Will my child’s tumor come back after treatment?

If we can completely remove the tumor surgically, it will most likely not come back. However, complete removal is not often possible due to the size, location or multiple sites of tumors. Remaining tumors may require further therapy and there is a risk of tumors coming back after treatment.

Epithelioid hemangioendothelioma: Reviewed by Cameron Trenor III, MD, © Boston Children's Hospital, 2015

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