Relapsed neuroblastoma refers to the return of neuroblastoma
in patients who have already undergone treatment for the disease. Approximately
half of children who are treated for high-risk neuroblastoma and achieve an
initial remission will have the disease come back.
In addition, in approximately 15 percent of children with high-risk
neuroblastoma, the tumor does not respond to initial treatment. These children
are said to have refractory neuroblastoma. The treatment approach for children
with refractory neuroblastoma is similar to that for children with relapsed
neuroblastoma.
Children and adolescents with relapsed or refractory
neuroblastoma are treated at Dana-Farber/Boston Children’s through the Neuroblastoma
Program,
one of the largest and most experienced pediatric neuroblastoma programs in the
world.
Our neuroblastoma specialists are known for treating
children with the most complex cases, as well as for their expertise in
delivering specialized treatments. We are one of only about 10
centers in the country, and the first and only center in New England, to offer MIBG therapy, an advanced treatment option for
neuroblastoma that delivers targeted radiation to kill neuroblastoma cells.
As with newly diagnosed neuroblastoma,
the symptoms of relapsed neuroblastoma can vary greatly, depending on the size
and location of the tumor and whether or not the tumor has spread.
While neuroblastoma usually begins in the abdomen,
especially in the tissues of the adrenal glands, it may also begin in nerve
tissues in the neck, chest or pelvis. These tumors often spread (metastasize)
to other areas of the body, including the lymph nodes, liver, bones and bone
marrow.
The symptoms of relapsed neuroblastoma can include:
- an
abdominal mass, either felt during an examination or seen as a swollen
abdomen
- enlarged
lymph nodes in the neck
- swelling
and bruising of the area around the eyes
- unexplained
fevers, bone pain or limping caused by bone or bone marrow involvement
- weakness
or paralysis caused by compression of the spinal cord by tumor
- weight
loss or poor appetite
- uncontrolled
eye or leg movements caused by an autoimmune response to the tumor can be asymptomatic and be
picked up by scans done as a routine check
To make a diagnosis of relapsed neuroblastoma, your doctor
may order a variety of tests, including:
- imaging
studies, such as a computerized tomography (CT or CAT) scan, magnetic
resonance imaging (MRI) scan or bone scan, to evaluate the tumor and
determine the extent and location of metastases
- a metaiodobenzylguanidine
(MIBG) scan, which uses a radioactive chemical that is absorbed by most
neuroblastomas, and a specialized scanner to locate the presence of the
neuroblastoma
- bone
marrow biopsy and/or aspiration, to determine whether neuroblastoma cells
have spread to the bone marrow
- urine
test to measure specific chemicals made by neuroblastoma cells
- blood
tests, including a complete blood count, blood chemistries, and kidney and
liver function tests
In some cases, your doctor may also order a tumor biopsy, in
which surgeons or interventional radiologists remove either a piece of the
tumor or the whole tumor, depending on tumor location and size. Pediatric
pathologists will analyze the tumor, and other important tests will be done to
determine the tumor biology, such as genetic studies to look for genes that may
be targeted using new drugs.
After all tests are completed, doctors will be able to
outline the best treatment options.
There is no standard treatment for relapsed neuroblastoma.
Rather, our treatment approach is personalized for each patient depending on
several factors, including:
- extent of relapse
- length of time from prior treatment
- type of prior treatment
Treatment options for relapsed neuroblastoma include:
- MIBG therapy, which uses a radioactive isotope that is
readily absorbed by most neuroblastomas and can be used
to detect neuroblastoma in the body or to deliver radiation in order to kill
the neuroblastoma cells
- chemotherapy, generally using combinations of
chemotherapy agents not used to treat newly diagnosed neuroblastoma
- immunotherapy, generally given in combination with chemotherapy, using Unituxin (dinutuximab), an antibody that binds to the surface of neuroblastoma cells
We also offer innovative clinical trials of experimental agents (Phase 1 or
2) for children with relapsed or recurrent neuroblastoma. Some of these were
launched by our own physicians, while others are available through our
participation in collaborative groups such as the New Approaches to Neuroblastoma Therapy
(NANT) consortium, the Children's Oncology Group (COG) and the Pediatric
Oncology Experimental Therapeutics Investigators' Consortium (POETIC).
Should you have questions
or need advice on whether a particular trial would be appropriate for your
child, email our clinical trials team at clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your
options.
At present, it is very difficult to cure relapsed high-risk neuroblastoma. For some patients, it can be managed as a chronic disease.
Research is a top priority at Dana-Farber/Boston Children's,
and our physicians work continuously to translate laboratory findings into
clinical therapies.
It’s possible that your child will be eligible to
participate in one of the Neuroblastoma Program’s current clinical trials. In addition to launching our
own clinical trials, we also offer the most Phase I studies in New England for
children whose disease has recurred through the Children's Oncology Group and
the New Approaches to Neuroblastoma Therapy (NANT) consortium.
Our current research efforts focus on improving established therapies
for newly diagnosed and relapsed or recurrent neuroblastoma, studying the
genetic causes of the disease and developing novel therapies. For instance,
recent laboratory and animal studies by researchers at Dana-Farber/Boston
Children's have focused on ways to counter MYCN amplification,
one of the most common genetic features of aggressive neuroblastomas.
Our researchers are also
teasing apart the relationship between neuroblastoma and a gene called ALK. We
are working on therapies that target this gene and may improve treatment for newly
diagnosed and relapsed neuroblastoma.