Daniel Rhee, M.D., M.P.H., pediatric surgeon and surgical director of the Pediatric Surgical Oncology Program at Johns Hopkins Children’s Center, discusses neuroblastoma, a complex cancer that requires a multidisciplinary treatment team. Neuroblastoma is rare, with only 700–800 new cases diagnosed in the U.S. per year, but it is the most common solid tumor seen in children. Dr. Rhee and his team are experienced clinicians dedicated to each child’s individual treatment plan.
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So, neuroblastoma is a, a type of tumor that arises from very early nerve cells. Uh these nerve cells instead of developing normally will proliferate in an uncontrolled fashion that leads to this cancer growth. These typically occur in the adrenal glands which are in the abdomen, but they can occur in other parts of the body such as the chest, the pelvis or even the neck. Uh and these follow along the sympathetic nerve chain which runs on both sides of the spinal column. These tumors can grow to be very large and they can spread to other locations such as the lymph nodes, the lungs, the liver or even the bone. And sometimes these tumors can even invade into the spinal canal. Overall, this is uh a rare tumor with only about 7 to 800 cases of neuroblastoma diagnosed each year in the United States. Uh However, is still, is still one of the most common tumors that we see in Children. These most commonly occur in Children that are less than to five. But occasionally we do see them in infants and even teenagers and on very rare occasions in young adults. So, neuroblastoma can present with a wide range of symptoms. Uh depending on the location of the tumor and the size and extent. Sometimes the only finding is a hard palpable mass. Other times it can present with uh symptoms such as pain, uh generalized weakness, fevers, lethargy, loss of appetite, and other nonspecific symptoms. Neuroblastoma can be hard to diagnose because many of these symptoms are also similar to other common childhood illnesses. And it's not uncommon for these masses to grow very big before a diagnosis is made. So, treatment for neuroblastoma depends largely on its involvement and extent as well as a patient's age and the underlying biology. The first steps for treatment is determining its risk and staging and this helps us determine how aggressive the tumor is as well as how we expect it to respond to different forms of treatment in order to do this, uh additional testing is needed including AC T scan or MRI uh additional lab tests as well as a tissue biopsy. Once this is done, a treatment plan can be made. Treatment often involves chemotherapy or surgery. In some cases, additional treatments such as radiation therapy, a bone marrow transplant or even immunotherapy is needed in general. The more aggressive the cancer is the more intensive the treatment needs to be interestingly, there are types of low risk neuroblastoma that are diagnosed either prenatally or very early on in life that we've learned that don't require any treatment at all. We actually observe these only following them with serial imaging and lab values and learn that most of these will basically mature into a noncancerous form and not require any type of surgery or chemotherapy. So, the surgeon covers many roles in the management of neuroblastoma. Uh First and foremost, the surgeon plays a role in establishing the safest and best way to make a diagnosis through a tissue biopsy. Of course, the surgeon is responsible for moving the tumor itself. These tumors can grow to be very large and they can also wrap themselves around very important structures in the body and the surgeon needs to be experienced to be able to remove this completely and safely without any long lasting complications towards a patient. Just as importantly, the surgeons need to be experienced and knowledgeable to know when a more reception is not necessary or even when surgery is not necessary at all. The surgeon also plays a role in other parts of the treatment including placing special lines that are needed to deliver chemotherapy, um feeding tubes to help maintain nutrition when they're needed. And also uh we perform procedures that assist with fertility preservation. So, neuroblastoma is a very complex type of cancer and there's been constant evolutionary understanding of how best to treat this cancer treatment for neuroblastma requires a multidisciplinary team. And that includes pediatric oncologists, pediatric surgeons, radiation oncologists, pathologists, diagnostic radiologists, as well as a team of nurse practitioners uh dedicated to Children with cancer and child life specialists at Johns Hopkins. We have a very large and experienced team of providers dedicated to the care of Children with cancer. We have specialists in neuroblastoma as well as leaders in the Chin's Oncology Group where most of the treatment protocols for neuroblastoma and other cancers are developed. I believe the team approach is essential. And as a team, we look at each patient with neuroblastoma individually and work with the family to ensure they're on the best pathway possible. This includes the best medical treatment pathway as well as the proper support so that they know they are not alone in this journey.
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