Surgical Treatment for Neuroblastoma

Most children with neuroblastoma will require surgery as part of their treatment plan. At Children’s Hospital of Philadelphia, your child will be treated by a team of specialists who work together to create a treatment plan specific to your child. You and your child will meet with the doctors and nurses repeatedly over the course of treatment to ensure your family has an opportunity to build a trusting relationship with the medical team.

Prior to treatment, your child’s neuroblastoma will be classified as low-, intermediate- or high-risk. This classification is based on tumor stage (localized or metastatic, involving vital structures or separate from them) and on careful analysis of tissue obtained during a biopsy. The risk level of the tumor is an important factor in determining the most effective treatment plan for your child.

Some children with low-risk neuroblastoma may be treated solely with surgery or with no intervention at all.

Children with intermediate- or high-risk neuroblastoma typically receive chemotherapy to shrink the tumor before surgery. Neuroblastomas tend to wrap around other structures within the body. Shrinking the tumor allows doctors to remove more of it and reduce the risk of damage to nearby healthy tissue.

Chemotherapy for high-risk disease typically involves five rounds (known as cycles) of treatment over the course of three to four months. During this time, stem cells will be taken from your child for later use in treatment. After the fourth or fifth round of chemotherapy but prior to surgery, doctors will order a CT or MRI scan to reassess the current extent of the tumor.

For most children with neuroblastoma, treatment includes surgery, sometimes simply a biopsy at first, with removal of remaining tumor later on. Although some neuroblastomas can be safely removed early in the course of therapy — which in very rare cases might be the only treatment necessary — most neuroblastomas have a very rich blood supply and tend to wrap around vital structures (such as major blood vessels), making it unsafe to try to remove them early in the course of treatment.

Some children with neuroblastoma will be required to undergo multiple surgical procedures, almost always performed under general anesthesia. For example, in addition to the operation during which the surgeon attempts to resect (remove) the primary (main portion of) tumor, your child might need to undergo other smaller procedures including a biopsy of the tumor, bone marrow biopsy, and placement of an intravenous access device for giving chemotherapy and drawing blood. These are often bundled together early in the course of therapy. These are generally very safe and straightforward procedures that we perform frequently at CHOP. Although we have a great deal of experience with them, we will discuss them with you ahead of time in great detail and will always make every effort to minimize your child’s discomfort and anxiety.

Children whose tumors cannot be removed early on usually require a biopsy to confirm the diagnosis. Although the biopsy can occasionally be performed using just a needle or a minimally invasive surgical approach, most patients need to have a standard surgical biopsy through a small incision under general anesthesia. This is a very safe operation that takes about an hour, causes mild discomfort, and entails an overnight stay in the hospital. Once the diagnosis is confirmed, the oncology team will recommend a course of therapy to help shrink the tumor and make it safer to be removed later.

After chemotherapy, and depending on the results of the MRI or CT scan, most patients with neuroblastoma will then need to undergo a more extensive surgery. The goal of the operation is to remove as much of the tumor as possible but also to avoid injury to all important organs and structures. About a week before the operation, you will meet with a surgical oncologist (a surgeon with expertise in removing neuroblastomas and other tumors) to discuss the details of the operation, the potential risks involved and any questions you may have. That same day you will also meet with someone from anesthesiology and your child will have some blood drawn for preoperative testing.

On the day of surgery, we will give your child medication to reduce their anxiety, and the nurses will bring them to the operating room. Once in the OR, after your child has gone to sleep, the clinical team will place IVs and administer medication for sedation and pain control.

Your child’s surgery to resect the primary tumor could take several hours. During the procedure you can wait in the OR family lounge. During the operation, staff from the Family Services team will check in with you periodically to provide updates. The specific type of surgery your child needs will depend on where the primary tumor is located and what other organs or anatomic structures are affected.

CHOP’s approach for high-risk abdominal tumors is to remove as much of the tumor as possible while avoiding injury to important organs and healthy tissue. In some cases, if the tumor has invaded the lymph nodes or other structures, those organs will need to be removed as well.

Because these tumors tend to wrap themselves around delicate anatomic structures, a neuroblastoma resection can be a long and difficult operation. At CHOP, you can be assured that your child’s surgeon will be an expert at performing these very challenging operations, and although they might need to be aggressive to remove as much tumor as possible, they will also always do everything possible to keep your child safe from harm. It is also important to remember that not all neuroblastomas can or need to be removed completely. Removing most of the tumor (debulking) is sometimes all that can be safely accomplished but also may be all that the patient needs.

The majority of neuroblastoma tumors are in the abdomen, but at times these tumors are located in other areas of the body. Tumors in the chest (thoracic) tend to be low- to intermediate-risk, so the surgeon might be able to take a less aggressive approach for these tumors compared to the approach for abdominal tumors. Depending on size and location, surgery may be performed using a minimally invasive (thoracoscopic) procedure, which entails multiple small incisions and video assistance. Some tumors, however, require a more traditional incision. In most cases, the surgical goal is to remove as much of the tumor as possible — at least 50%. If the tumor is attached to important blood vessels or other structures, it may be safer to leave some of the tumor behind rather than risk damaging healthy organs. Less often, the primary tumor site may be in the pelvis or neck (cervical tumors). These also have slightly different risks and surgical approaches. Cervical tumors tend to be low- to intermediate-risk but may be close to major blood vessels. Pelvic tumors are also lower risk but often are close to important nerves and organs such as the bowel or bladder. In most pelvic and cervical procedures, the surgeon will aim to remove at least half of the tumor but may leave some behind to avoid damage to important structures.

Once the surgery is complete, your child’s surgeon will meet with you to provide more details and answer any questions you have. Most children will stabilize in the recovery room. In a few cases, when the surgery is more complex, your child may be sent to the Pediatric Intensive Care Unit (PICU) to recover. Family Services will escort you there to see them. Your child will typically be cleared to go home when certain milestones have been met, typically within five to seven days after surgery.

After your child’s surgery, doctors will order a series of imaging and other tests to see how much, if any, of the neuroblastoma remains. You’ll receive a schedule of regular follow-up appointments.

Follow-up care is important so doctors can monitor your child’s progress and check for potential relapses. It also gives you an opportunity to discuss your concerns and get answers to any questions you may have as your child returns to school and other activities. During this time, it’s also important that you keep an eye out for any potentially troubling symptoms and share them with your child’s doctor right away.

Children with high-risk disease will usually resume treatment approximately one week after surgery. Your child will receive chemotherapy to treat any remaining tumor and then will have tests to determine how the neuroblastoma has responded to therapy. If there has been a good response, your child will go on to receive high doses of chemotherapy as well as infusions of the previously harvested stem cells.

After recovery from the high-dose therapy, your child will also receive radiation treatment to target any remaining tumor cells. Proton therapy may be recommended by the radiation team. With proton therapy, doctors can more accurately direct the radiation to your child’s tumor, which means less damage to healthy tissue and fewer side effects. Through a collaboration with Penn Medicine, CHOP is able to offer pediatric proton therapy at the Roberts Proton Therapy Center.

In the final phase of high-risk treatment, your child will likely receive approximately six months of therapy that employs the body’s own immune system to treat any remaining cancer cells.

Unfortunately, high-risk neuroblastoma doesn’t always respond to treatment or recurs during or after treatment. This is known as relapsed or refractory neuroblastoma. CHOP has a multidisciplinary approach to treat children with relapsed or refractory neuroblastoma through its Refractory Neuroblastoma Program. The refractory neuroblastoma treatment group is comprised of pediatric oncologists, oncology nurse practitioners, social workers, a case manager, an outpatient scheduler, and clinical research assistants who specialize in working with children who have neuroblastoma.

Even when neuroblastoma is successfully cured, children treated for cancer sometimes develop complications years later. CHOP’s Cancer Survivorship Program can provide your family with information about the potential long-term effects of the specific neuroblastoma treatment your child received, including ways of monitoring and treating these effects.