What Is Neuroblastoma?
Neuroblastoma (neuro = nerve, blastoma = collection of tumor cells) is a type of cancer that begins in the
embryonic cells which normally develop into parts of our nervous system. These cells are called neuroblasts.
A tumor arising from these cells is called a neuroblastoma. The cause of neuroblastoma is not known. We do
know that it is not hereditary, and does not result from injury and is not infectious.
Neuroblastoma is responsible for 8-10% of all childhood cancers. Although it is only the 5th most
common cancer in childhood, it is responsible for a disproportionate number of deaths due to cancer
in children. Neuroblastoma usually occurs in children under 5. It is the most common tumor in babies
under 1 year of age. Although it is rare, neuroblastoma can occur in older children and adults.
Every year throughout North America, hundreds children might be diagnosed with neuroblastoma.
Sixty percent of children diagnosed over the age of 1 have widespread (metastatic) disease at diagnosis.
Over the last 20 years there has been modest improvement, due to research, in the prognosis for children
diagnosed at less than 1 year of age with neuroblastoma, however, despite research initiatives, there has
been little improvement in the prognosis for older children with widespread disease at diagnosis. The vast
majority of these children die.
Each child may experience symptoms differently. The symptoms of neuroblastoma vary greatly depending on size, location, and spread of the tumor. The most common symptoms include:
- an abdominal mass, either felt during an examination or seen as a swollen abdomen
- tumors in the face or head that cause swelling and bruising of the area around the eyes and uncontrolled eye movement
- compression of the kidney or bladder by the tumor that causes changes in urination
- Pain, limping, paralysis, or weakness caused by bone or bone marrow involvement, or spinal cord tumor growth
- cough or shortness of breath caused by a tumor in the chest
- diarrhea caused by a substance produced by the tumor
- loss of balance and dizziness
- fever
- high blood pressure and increased heart rate, depending on location of the tumor and the organs the tumor compresses
- weight loss or poor appetite
About 40% of neuroblastomas develop in the adrenal gland which is just above the kidney. The rest begin in a chain of nerve tissue that runs on each side of the spine. Tumors will then develop in the neck, chest, pelvis or abdomen.
Symptoms also result from the tumor spreading to different parts of the body. In 70% of patients the original
tumor has already spread to other parts of the body before any symptoms are seen. Neuroblastoma may spread
to bone, bone marrow, liver, skin and lymph nodes. Ultrasound, CT Scans, liver scans, bone scans and MIBG scans
are all used to diagnose neuroblastoma and to detect tumor locations throughout the body. Urine and blood tests
are also done. A biopsy of the tumor is usually taken to confirm the diagnosis.
When neuroblastoma is diagnosed it is classified as being in one of 5 stages. The stage of disease tells us how far
the disease has spread and determines what the best plan of treatment should be.
Stage 1: This is when a single tumor has been detected early and no disease has spread. The tumor is removed
surgically usually with no need for chemotherapy.
Stage 2: This applies to a single tumor site that cannot be completely surgically removed. Disease may also be
present in some lymph nodes local to the tumor. Chemotherapy will usually be required.
Stage 3: This refers to a large tumor that has spread across the body. Surgery is not an option at this point and
chemotherapy is needed to reduce the size of the tumor.
Stage 4: The tumor can be of any size, but is usually very large, often with several further tumors detected. The
disease has metastasized to far reaching parts of the body such as lymph nodes, bones and the brain.
Stage 4s: This is a special stage that is found in infants under the age of one. It is a small tumor that has spread to
other parts of the body, but usually regresses naturally without any intervention.
Sometimes surgery can be done to remove the tumor. If the tumor cannot be safely removed then chemotherapy may
be used to help shrink the tumor so that it can possibly be removed. Chemotherapy is used when the tumor cells have
spread throughout the body. Bone Marrow Transplant is often the treatment offered to patients with widespread disease
and a poor prognosis once the neuroblastoma is under control.
The prognosis for neuroblastoma depends on the age of the child and the stage of the disease at diagnosis. The younger
the child is at diagnosis the greater the chance for cure is. If there is no sign of disease recurrence 5 years after diagnosis
the chances are very good that the child has been cured. The current 5 year survival rate after bone marrow transplant for children with neuroblastoma is 20-25%.
Every 16 hours a child with neuroblastoma dies. There is no known cure for relapsed neuroblastoma. Nearly 70% of those
children first diagnosed with neuroblastoma have disease that has already metastasized or spread to other parts of the body. When disease has spread at diagnosis and a child is over the age of 2, there is less than a 30% chance of survival.
Information taken from:
http://www.jamesfund.com/about-neuroblastoma
http://www.familiesagainstneuroblastoma.org/FamiliesAgainstNeuroblastoma_What_is_Neuroblastoma.html
http://www.childrenshospital.org/az/Site1084/mainpageS1084P1.html
embryonic cells which normally develop into parts of our nervous system. These cells are called neuroblasts.
A tumor arising from these cells is called a neuroblastoma. The cause of neuroblastoma is not known. We do
know that it is not hereditary, and does not result from injury and is not infectious.
Neuroblastoma is responsible for 8-10% of all childhood cancers. Although it is only the 5th most
common cancer in childhood, it is responsible for a disproportionate number of deaths due to cancer
in children. Neuroblastoma usually occurs in children under 5. It is the most common tumor in babies
under 1 year of age. Although it is rare, neuroblastoma can occur in older children and adults.
Every year throughout North America, hundreds children might be diagnosed with neuroblastoma.
Sixty percent of children diagnosed over the age of 1 have widespread (metastatic) disease at diagnosis.
Over the last 20 years there has been modest improvement, due to research, in the prognosis for children
diagnosed at less than 1 year of age with neuroblastoma, however, despite research initiatives, there has
been little improvement in the prognosis for older children with widespread disease at diagnosis. The vast
majority of these children die.
Each child may experience symptoms differently. The symptoms of neuroblastoma vary greatly depending on size, location, and spread of the tumor. The most common symptoms include:
- an abdominal mass, either felt during an examination or seen as a swollen abdomen
- tumors in the face or head that cause swelling and bruising of the area around the eyes and uncontrolled eye movement
- compression of the kidney or bladder by the tumor that causes changes in urination
- Pain, limping, paralysis, or weakness caused by bone or bone marrow involvement, or spinal cord tumor growth
- cough or shortness of breath caused by a tumor in the chest
- diarrhea caused by a substance produced by the tumor
- loss of balance and dizziness
- fever
- high blood pressure and increased heart rate, depending on location of the tumor and the organs the tumor compresses
- weight loss or poor appetite
About 40% of neuroblastomas develop in the adrenal gland which is just above the kidney. The rest begin in a chain of nerve tissue that runs on each side of the spine. Tumors will then develop in the neck, chest, pelvis or abdomen.
Symptoms also result from the tumor spreading to different parts of the body. In 70% of patients the original
tumor has already spread to other parts of the body before any symptoms are seen. Neuroblastoma may spread
to bone, bone marrow, liver, skin and lymph nodes. Ultrasound, CT Scans, liver scans, bone scans and MIBG scans
are all used to diagnose neuroblastoma and to detect tumor locations throughout the body. Urine and blood tests
are also done. A biopsy of the tumor is usually taken to confirm the diagnosis.
When neuroblastoma is diagnosed it is classified as being in one of 5 stages. The stage of disease tells us how far
the disease has spread and determines what the best plan of treatment should be.
Stage 1: This is when a single tumor has been detected early and no disease has spread. The tumor is removed
surgically usually with no need for chemotherapy.
Stage 2: This applies to a single tumor site that cannot be completely surgically removed. Disease may also be
present in some lymph nodes local to the tumor. Chemotherapy will usually be required.
Stage 3: This refers to a large tumor that has spread across the body. Surgery is not an option at this point and
chemotherapy is needed to reduce the size of the tumor.
Stage 4: The tumor can be of any size, but is usually very large, often with several further tumors detected. The
disease has metastasized to far reaching parts of the body such as lymph nodes, bones and the brain.
Stage 4s: This is a special stage that is found in infants under the age of one. It is a small tumor that has spread to
other parts of the body, but usually regresses naturally without any intervention.
Sometimes surgery can be done to remove the tumor. If the tumor cannot be safely removed then chemotherapy may
be used to help shrink the tumor so that it can possibly be removed. Chemotherapy is used when the tumor cells have
spread throughout the body. Bone Marrow Transplant is often the treatment offered to patients with widespread disease
and a poor prognosis once the neuroblastoma is under control.
The prognosis for neuroblastoma depends on the age of the child and the stage of the disease at diagnosis. The younger
the child is at diagnosis the greater the chance for cure is. If there is no sign of disease recurrence 5 years after diagnosis
the chances are very good that the child has been cured. The current 5 year survival rate after bone marrow transplant for children with neuroblastoma is 20-25%.
Every 16 hours a child with neuroblastoma dies. There is no known cure for relapsed neuroblastoma. Nearly 70% of those
children first diagnosed with neuroblastoma have disease that has already metastasized or spread to other parts of the body. When disease has spread at diagnosis and a child is over the age of 2, there is less than a 30% chance of survival.
Information taken from:
http://www.jamesfund.com/about-neuroblastoma
http://www.familiesagainstneuroblastoma.org/FamiliesAgainstNeuroblastoma_What_is_Neuroblastoma.html
http://www.childrenshospital.org/az/Site1084/mainpageS1084P1.html
FAN Image
Neuroblastoma Statistics for Canada
Neuroblastoma is the MOST COMMON and MOST DEADLY solid tumour that occurs in children outside of the brain (Hansford et al, 2007; Maris, 2007; Mueller and Matthay, 2009; Chang and Hsu, 2010; Smith et al, 2010). Overall, it is the most common tumour diagnosed in the first year of life (Maris, 2010).
Approximately 50-70 new cases of neuroblastoma are diagnosed every year in Canada and in about 50-70% of these cases the disease has already metastasized at the time it is found. This is the highest rate of metastasis for all paediatric cancers (Canadian Cancer Society, 2008).
Neuroblastoma accounts for 15% of childhood cancer mortality (Maris, 2007, p. 2106; Mueller and Matthay, 2009, p. 431) and has the lowest observed survival proportions (OSP) out of all paediatric cancers (72% for neuroblastoma) (Canadian Cancer Society, 2009, p. 49). “Tumour groups that have disappointing survival rates include metastatic solid tumours such as Ewing’s
sarcoma, rhabdomyosarcoma, osteosarcoma, and neuroblastoma” (Canadian Cancer Society, 2008, p. 63).
“High-risk patients with evidence of metastasis, however, have an overall survival (OS) rate of LESS THAN 40%, despite intensive multimodality treatment” (Mueller and Matthay, 2009, p. 431). This survival rate has “shown only modest
improvement, despite dramatic escalations in the intensity of therapy provided” (Maris, 2010, 2202).
Approximately 50-60% of high-risk neuroblastoma patients will have a relapse. There is NO CURE for relapsed neuroblastoma (Maris, 2010).
Immunotherapy is showing great promise for high-risk neuroblastoma and involves activating the body’s own immune system to kill cancer cells. In a recent trial, two-year survival rates increased by 20% for those children who had the ch14.18 antibody (Yu et al., 2009). Immunotherapy is now a new standard in the treatment of high-risk neuroblastoma.
“Treatment of older children with widely disseminated neuroblastoma (stage 4) remains one of the greatest challenges for paediatric oncologists” (Maris, 2007, p. 2111).
“Neuroblastoma has the highest rate of spontaneous regression observed in human cancers” (Brodeur, 2003, p. 209).
Patients who are classified within the low-risk category of the disease have an overall survival probability of 85-92% (Maris, 2007, p. 2111).
As few as 10 neuroblastoma tumour-initiating cells transplanted into an immune compromised mouse can form tumours which are both aggressive and metastatic (Hansford et al., 2007 and Smith et al., 2010).
“It is rare in adults and more than 96% of patients diagnosed with neuroblastoma are less than 10 years of age” (Chang and Hsu, 2010, p. 555).
Neuroblastoma is one of the most expensive cancers to treat (Canadian Cancer Society, 2010, p 86). The treatment regime for high-risk neuroblastoma includes chemotherapy, surgery, high-dose chemotherapy with stem cell transplant, radiation, and immunotherapy. Patients must go through all these modalities, typically experience a wide-array of complications, and are
monitored for years after completion of treatment (Kushner and Cheung, 2005, p. 2215).
Taken from Neuroblastoma Canada: http://www.neuroblastoma.ca/neuroblastoma/statistics
Childhood Cancer in Canada
There are about 10,000 children living with cancer in Canada today.
Each year, about 1500 cases are diagnosed.Because of significant advances in therapy, 78% of these children will survive 5 years or more, an increase of almost 46% since the early 1960s.
More than 70 percent of children diagnosed with cancer become long-term survivors and the majority of them are considered cured. However, long-term effects of surviving the treatments for childhood cancer can affect these children's futures.
In the early 1950s, less than 10 percent of childhood cancer patients could be cured.
Leukemias, tumors of the brain and nervous system, the lymphatic system, kidneys, bones and muscles are the most common childhood cancers.
In Canada, childhood cancer remains responsible for more deaths from one year through adolescence than any other disease; more deaths than asthma, diabetes, cystic fibrosis and AIDS combined.
Childhood cancers have close to a 75% cure rate, with leukemia leading the success charge with close to 90% overall cure rate.
About one in four children who are diagnosed with cancer will die of the disease.
With a mortality rate of close to 25%, Canada loses tens of thousands of years of potential life each year to childhood cancer.
In terms of potential life saved, childhood cancer rank second only to breast cancer.
Childhood cancers differ from adult cancers. Adults are most affected by breast, lung, prostate, bowel and bladder cancers. Children are most affected by acute leukemia, tumours of the brain and nervous system, the lymphatic system, kidneys, bones and muscles.
Leukemia is the most commonly diagnosed cancer in children, comprising some 30% of the total new cases diagnosed each year. Acute lymphoblastic leukemia (ALL) is the most common form and comprises about 75% of these cases.
Peak incidence is at two to three years of age, with boys 20% more likely to contract the disease. Survival rate is now 95%, with a 30% relapse rate.
Lymphomas and cancers of the central nervous system are the second most diagnosed children's cancer, at 17% of total cancers diagnosed.
Sarcomas, or cancers of the muscles, bones, nerves, fat, blood or connective tissue are next at 12%, with boys 25-30% more likely to contract this form of cancer.
Neuroblastomas, which represent 95% of tumors in the sympathetic nervous system, represent 5% of all childhood cancers.
Taken from Childhood Cancer Canada http://childhoodcancer.ca/education/facts_figures
****************************************************************************************************
"Every year in Canada, cancer is diagnosed in about 850 children (143 per million in 2004) between birth and 14 years of age” (Ellison et al, 2009, p. 422; Canadian Cancer Society, 2008).
“The rate of death from childhood cancer has decreased substantially but is still about 135 deaths each year (24.6 per million in 2004)” (Ellison et al, 2009, p. 422; Canadian Cancer Society, 2008).
Out of all of the cancers diagnosed in Canada in a year, childhood cancers account for a little over half of one percent (Canadian Cancer Society, 2008).
“Survivors of childhood cancer are at substantial risk for health problems, including late sequelae of cancer treatment and chronic psychological and cognitive impairments. The latter can hinder both their psychosocial adjustment after treatment and their health and well-being over the long term” (Ellison et al, 2009, p. 422).
“Little is known about the cause of childhood cancers…Opportunities to prevent cancer in children are therefore limited. This is especially so because screening tests are lacking for most childhood cancers” (Ellison et al, 2009, p. 422).
“Five-year survival rates for childhood cancers have improved considerably in Canada, from 71% in the late 1980s4 to 82% in the early 2000s” (Ellison et al, 2009, p. 422).
“Improving survival in childhood cancer (now at 82%), places increasing need for long term follow-up of late effects” (Canadian Cancer Society, 2008, p. 2). “An estimated two-thirds of survivors have at least one chronic or late-occurring effect from their cancer therapy, while up to one-third have a major, serious or life threatening complication. Endocrine and metabolic complications are the most prevalent late effects among childhood cancer survivors, followed by sensory problems, neurocognitive impairment, cardiopulmonary dysfunction, gastrointestinal disorders and secondary malignant neoplasms. Survivors are also found to be at an increased risk of early death up to 25 years after diagnosis, owing mainly to a relapse of the primary cancer in the early years following the completion of therapy”. (Canadian Cancer Society, 2008, p. 63, and Ellison et al, 2009)
“Definitive diagnosis and treatment for children with cancer is available at one of 17 specialized pediatric cancer centres in Canada” (Canadian Cancer Society, 2008).
“Cancers in children differ from those occurring in adults in both their site of origin and their behaviour. The majority of cancers in adults are carcinomas which start in the glands or tissues that line organs such as the breast, lung, prostate or colon. In children, carcinomas are very rare. Tumours in children have short latency periods, often grow rapidly and are aggressive, invasive and frequently spread to other parts of the body” (Canadian Cancer Society, 2008, p. 61).
Taken from Neuroblastoma Canada: http://www.neuroblastoma.ca/neuroblastoma/statistics
Each year, about 1500 cases are diagnosed.Because of significant advances in therapy, 78% of these children will survive 5 years or more, an increase of almost 46% since the early 1960s.
More than 70 percent of children diagnosed with cancer become long-term survivors and the majority of them are considered cured. However, long-term effects of surviving the treatments for childhood cancer can affect these children's futures.
In the early 1950s, less than 10 percent of childhood cancer patients could be cured.
Leukemias, tumors of the brain and nervous system, the lymphatic system, kidneys, bones and muscles are the most common childhood cancers.
In Canada, childhood cancer remains responsible for more deaths from one year through adolescence than any other disease; more deaths than asthma, diabetes, cystic fibrosis and AIDS combined.
Childhood cancers have close to a 75% cure rate, with leukemia leading the success charge with close to 90% overall cure rate.
About one in four children who are diagnosed with cancer will die of the disease.
With a mortality rate of close to 25%, Canada loses tens of thousands of years of potential life each year to childhood cancer.
In terms of potential life saved, childhood cancer rank second only to breast cancer.
Childhood cancers differ from adult cancers. Adults are most affected by breast, lung, prostate, bowel and bladder cancers. Children are most affected by acute leukemia, tumours of the brain and nervous system, the lymphatic system, kidneys, bones and muscles.
Leukemia is the most commonly diagnosed cancer in children, comprising some 30% of the total new cases diagnosed each year. Acute lymphoblastic leukemia (ALL) is the most common form and comprises about 75% of these cases.
Peak incidence is at two to three years of age, with boys 20% more likely to contract the disease. Survival rate is now 95%, with a 30% relapse rate.
Lymphomas and cancers of the central nervous system are the second most diagnosed children's cancer, at 17% of total cancers diagnosed.
Sarcomas, or cancers of the muscles, bones, nerves, fat, blood or connective tissue are next at 12%, with boys 25-30% more likely to contract this form of cancer.
Neuroblastomas, which represent 95% of tumors in the sympathetic nervous system, represent 5% of all childhood cancers.
Taken from Childhood Cancer Canada http://childhoodcancer.ca/education/facts_figures
****************************************************************************************************
"Every year in Canada, cancer is diagnosed in about 850 children (143 per million in 2004) between birth and 14 years of age” (Ellison et al, 2009, p. 422; Canadian Cancer Society, 2008).
“The rate of death from childhood cancer has decreased substantially but is still about 135 deaths each year (24.6 per million in 2004)” (Ellison et al, 2009, p. 422; Canadian Cancer Society, 2008).
Out of all of the cancers diagnosed in Canada in a year, childhood cancers account for a little over half of one percent (Canadian Cancer Society, 2008).
“Survivors of childhood cancer are at substantial risk for health problems, including late sequelae of cancer treatment and chronic psychological and cognitive impairments. The latter can hinder both their psychosocial adjustment after treatment and their health and well-being over the long term” (Ellison et al, 2009, p. 422).
“Little is known about the cause of childhood cancers…Opportunities to prevent cancer in children are therefore limited. This is especially so because screening tests are lacking for most childhood cancers” (Ellison et al, 2009, p. 422).
“Five-year survival rates for childhood cancers have improved considerably in Canada, from 71% in the late 1980s4 to 82% in the early 2000s” (Ellison et al, 2009, p. 422).
“Improving survival in childhood cancer (now at 82%), places increasing need for long term follow-up of late effects” (Canadian Cancer Society, 2008, p. 2). “An estimated two-thirds of survivors have at least one chronic or late-occurring effect from their cancer therapy, while up to one-third have a major, serious or life threatening complication. Endocrine and metabolic complications are the most prevalent late effects among childhood cancer survivors, followed by sensory problems, neurocognitive impairment, cardiopulmonary dysfunction, gastrointestinal disorders and secondary malignant neoplasms. Survivors are also found to be at an increased risk of early death up to 25 years after diagnosis, owing mainly to a relapse of the primary cancer in the early years following the completion of therapy”. (Canadian Cancer Society, 2008, p. 63, and Ellison et al, 2009)
“Definitive diagnosis and treatment for children with cancer is available at one of 17 specialized pediatric cancer centres in Canada” (Canadian Cancer Society, 2008).
“Cancers in children differ from those occurring in adults in both their site of origin and their behaviour. The majority of cancers in adults are carcinomas which start in the glands or tissues that line organs such as the breast, lung, prostate or colon. In children, carcinomas are very rare. Tumours in children have short latency periods, often grow rapidly and are aggressive, invasive and frequently spread to other parts of the body” (Canadian Cancer Society, 2008, p. 61).
Taken from Neuroblastoma Canada: http://www.neuroblastoma.ca/neuroblastoma/statistics
