Childhood Cancer Awareness Month

Posted on September 05 2018

September is Childhood Cancer Awareness Month and today, we will be discussing this, in hopes to bring awareness regarding this horrific disease. 

Each year, more than 15,000 children and teenagers are diagnosed with cancer - that equates to approximately 42 per day, and that is heart wrenching. Though the five year survival rate for childhood cancers has reached 80%, nearly 2,000 children under age 19 pass away each year, making cancer the leading killer of children by disease. What's even more surprising is that these statistics are just within the United States. Over 300,000 children and teens have been diagnosed worldwide.

Childhood cancer remains a large problem because children's cancer cannot be treated exactly like adult cancers - which is where most of the federal research funding goes. Current treatments are toxic, can affect a child's development, and can be decades old. To treat childhood cancer in the best way possible, there needs to be specialized treatments just for kids.

Hospitals across the Country, especially St. Jude's Children's Research Hospital, are formulating and researching new state-of-the-art treatments to find cures, combat the various cancers, and drastically improve the survival rates for children and young adults. The causes of childhood cancer are largely unknown, but scientists continue to study what causes it, in order to understand what treatments work best.

Of course, treatment is key to fighting any type of cancer, but it is especially critical in childhood cancer patients. Many childhood cancer survivors in the United States suffer from lifelong damage to their organs, mental health, and more. The medical community is trying to understand how treatments affect children long-term, so that they can prevent late effects.

Types of Childhood Cancers

Brain and Neural Tumors

These types of cancer affect the brain, spine, and nervous system.

Brain Tumors

Brain tumors are tumors that grow inside of the skull, and they are the most common types of childhood cancer in the United States.

Treatment for children depends on where the tumor is located, and what type of cancer it is. The treatment can be different than adults, and may include surgical tumor removal, stem cell transplants, chemotherapy, and/or radiation therapy.

While these treatments can provide recovery, research states that there needs to still be better and safer treatments. Some late term effects from treatment and tumor removal include: seizures, hearing or vision loss or impairment, learning disabilities, and more.

The five year survival rate can range anywhere from 30 - 95% based on the type of cancer the child has.

Neuroblastoma

Neuroblastoma is a cancer that forms in a child's nerve tissue. It can form in the adrenal glands, neck, chest, or spinal cord. Sometimes, the disease can even begin growing before a child is born. Nearly 90% of the children diagnosed with this disease are under 5 years of age.

Treatment for this type of cancer includes: surgery, radiation therapy, chemotherapy, immunotherapy, stem cell transplants, or a combination of all of them. Even if a child has no evidence of Neuroblastoma left, they still need to go through all of the treatment, which can take over a year. 

Children who survive neuroblastoma can suffer from a variety of late effects, including: hearing loss, inner ear damage, neurological disorders of the eye, infertility, and more. 

The five year survival rate for children with low risk neuroblastoma is greater than 95%, but for children in the high risk group, the five year survival rate can be as low as 40 to 50 percent. The long-term survival rate is sadly even lower, at 15%. 

Retinoblastoma

Retinoblastoma is a cancer which occurs in the retina of a child's eye, and most often affects children who are under the age of six. Approximately 200 to 300 children are diagnosed with retinoblastoma each year in the United States. Many families discover that their child may have retinoblastoma when the pupil of their child's eye has a white glow that is seen in photographs. 

Children with retinoblastoma may undergo laser surgery, radiation therapy, chemotherapy, or in severe cases, the eye may be removed. Children can also receive cryotherapy - or, freezing therapy - in conjunction with laser therapy and chemotherapy. 

Why do we need better and safer treatments?

Well, long term effects from treatment may include blindness, vision impairment, reduced kidney function, hearing loss, delays in growth development, increased risk of other cancers, and more. 

Currently, the five year survival rate for children with retinoblastoma is 97%. However, survival rates are significantly lower in children whose cancer has spread outside of the retina.

Leukemia

Leukemia and other diseases of the blood and bone marrow usually affect red blood cells, white blood cells, and platelets. It is said that Leukemia is one of the most common types of cancer in children, teenagers, and adults. There are four common types of Leukemia found in childhood cancer, and they are:

1. Acute Lymphoblastic Leukemia: Or, ALL. ALL is a fast growing form of Leukemia that occurs when the bone marrow makes too many immature lymphocytes, which are a type of white blood cell. White blood cells help to fight infection and protect the body against disease. These immature white blood cells crowd out the normal white blood cells. Without enough normal white blood cells, the body has a harder time fighting infections. ALL causes the lymphocytes to build up in the liver, spleen, and lymph nodes.

ALL is the most common type of blood cancer in children. It most often occurs between the ages of 3 years old and 5 years old, and affects slightly more boys than girls. Approximately 3,000 people younger than age 20 are found to have ALL each year in the United States. About 98% of children with ALL go into remission within weeks after starting treatment, and about 90% of those children can be cured. Patients are considered cured after 10 years in remission. 

Symptoms of ALL to watch for are: Frequent infections, fever, easy bruising, bleeding that is hard to stop, flat or dark red skin spots due to bleeding under the skin, pain the bones or joints, lumps in the neck, underarm, stomach or groin, pain or fullness below the ribs, weakness and fatigue, paleness, loss of appetite, and shortness of breath.

Treatment for ALL includes three phases: Induction - to kill the leukemia cells in the blood and bone marrow, and put the disease into remission. Consolidation/Intensification - to rid the body of any remaining cells that could begin to grow and cause the leukemia to return. Maintenance - to destroy any cancer cells that might have survived the first two phases.

Four types of treatment may be used during any of these treatment phases, including: chemotherapy, stem cell transplant, radiation therapy, and targeted therapy.

2. Acute Myeloid Leukemia: Or, AML. AML is a type of leukemia in which the bone marrow makes a large number of abnormal blood cells that do not become healthy cells. These abnormal cells crowd out the normal ones, so the body has a harder time fighting off infection. Approximately 500 children are found to have AML in the United States each year, as it is much more common in adults. However, it is the most common second cancer among children treated for other cancers. AML is also seen more often in people who are exposed to large amounts of radiation, and some types of chemicals.

Symptoms of AML: Fever, infection, easy bruising and bleeding, frequent nosebleeds, bleeding that is difficult to stop, even from a small cut, pain in the bones or joints, swollen glands, and poor appetite.

Treatment for AML is primarily chemotherapy. Doctors look at several factors to decide which medications to use and how aggressive treatment should be. Some of these factors include the child's age, and the white blood cell count before treatment. Treatment for AML includes two phases: Induction with possible Central Nervous System Prophylaxis, and Consolidation/intensification/post-induction phase. For children with AML, the 5 year survival rate now ranges between 60-70 percent.

3. Juvenile Myelomonocytic Leukemia: Or, JMML. JMML is a rare and serious form of childhood leukemia. JMML occurs when too many blood stem cells become white blood cells called monocytes and myelocytes. Some of these cells mature into normal white blood cells, but many of them never fully mature. These immature white blood cells are called blasts. Over time, they crowd out the healthy red blood cells and platelets in the bone marrow. This may cause infection, anemia, or bleeding problems. JMML can also spread from the bone marrow to other areas of the body, such as the skin, lungs, liver, or intestines.

JMML occurs most often in children younger than 4 years old. 10% of all cases develop in infants younger than 3 months. One to two children out of a million are found to have JMML each year, and the disease accounts for 1.6% of all blood related cancers. Boys are twice as likely to get JMML than girls.

Symptoms of JMML to watch for: fever for unknown reasons, infections, such as bronchitis or tonsillitis, fatigue, easy bruising or bleeding of the skin, nose, and mouth, red skin rash, or tiny, red dots on the skin, painless swelling of the lymph nodes in the neck, underarm, stomach or groin, pain or feeling of fullness below the ribs, dry cough, trouble gaining weight, poor appetite, failure to thrive, irritability, enlarged spleen or liver, and anemia.

Currently, the only treatment for JMML that offers hope of a long term cure is stem cell transplants. This transplant uses healthy bone marrow stem cells or umbilical cord stem cells donated by another person. This type of transplant is called an allogeneic stem cell transplant. The stem cell transplant should be done as soon as possible after diagnosis because successful transplant rated are highest in younger children.

Sadly, for children with JMML who don't receive treatment, only 5 to 10 percent will recover. With allogenic stem cell transplant, many children with JMML are cured. In approximately 35 to 40 percent of children who achieve remission with an allogenic stem cell transplant, the JMML comes back within the first year. A second stem cell transplant has been shown to be effective for those patients.

4. Chronic Myelogenous Leukemia: Or, CML. CML is a form of leukemia that occurs when too many bone marrow stem cells become a type of white blood cell called granulocytes. Some of these never become mature white blood cells. While far more common in adults, CML is rare in children and accounts for about 1% of all childhood leukemias. CML can develop over a period of months or years.

Symptoms of CML: Anemia, bleeding and/or bruising, bone and joint pain, recurrent fevers or infections, abdominal pain, swollen lymph nodes, difficulty breathing.

Treatment for CML as of now is targeted chemotherapy, or stem cell transplants. This type of leukemia has a 5 year survival rate of approximately 60 to 80 percent, and through research and funding, that number is hoped to increase.

Lymphoma

 

Lymphoma is the third most common type of childhood cancer. It forms in the lymphatic system, which is part of the body's immune system. There are 2 main categories of lymphoma: Hodgkin Lymphoma and Non-Hodgkin Lymphoma. Doctors determine the type of lymphoma by looking at the cancer cells under a microscope.

Hodgkin Lymphoma

In Hodgkin Lymphoma, a certain kind of cell - called the Reed Sternberg cell - begins to reproduce uncontrollably. It's the overabundance of this specific type of cell that distinguishes Hodgkin Lymphoma from Non-Hodgkin Lymphoma.

Approximately 6% of childhood cancers are Hodgkin Lymphoma and it's most often found in adolescents ages 15 to 19. Treatment for this cancer may include chemotherapy, radiation, targeted therapy, surgery, or a combination of these options.

Symptoms include: swollen, but not sore, lymph nodes in the neck, chest, armpit, or groin, extreme fatigue, loss of appetite, weight loss, fever, night sweats, and itchy skin. 

Non-Hodgkin Lymphoma

There are 3 main types of childhood Non-Hodgkin Lymphomas, or NHLs. The types are based on the cell type and size of the cancer. Lymphoblastic Lymphoma accounts for 20% of childhood NHLs. Mature B-Cell Lymphoma, including Burkitt Lymphoma/Leukemia, accounts for about 51 to 62 percent of NHLs. Lastly, Large Cell Lymphoma accounts for approximately 10% of NHLs.

Symptoms of NHL include: coughing, wheezing, unexplained weight loss, night sweats, a painless lump or swelling of the testicle(s), and trouble swallowing.

Non-Hodgkin Lymphoma is more common than Hodgkin Lymphoma in children up to age 14, with about 500 cases diagnosed in the United States each year. Treatment for Non-Hodgkin Lymphoma depends on the type of lymphoma, and options may include chemotherapy, targeted therapy, surgery, or stem cell transplant.

So, why do we need better or safer treatments? The 5 year survival rate for children and adolescents with Hodgkin Lymphoma is 95%. The 5 year survival rate for Non-Hodgkin Lymphoma varies from 60 to over 90 percent, again, depending on the type of lymphoma, the location and size of the tumor, and other factors.

With more children surviving lymphomas, doctors are now focused on finding safer, less toxic treatments to reduce the risk of late effects, such as heart and cognitive issues, growth development and infertility.

 Sarcomas

Sarcomas are cancerous tumors that develop in the soft tissue and bone. There are 4 types of common sarcomas that we will explain below. 

Osteosarcoma

The most common bone cancer, osteosarcoma tends to occur in teenagers. The cancer usually begins in osteoblasts, which are a type of bone cell that becomes new bone tissue. 

Standard treatment for teenagers and children who have osteosarcoma typically includes: chemotherapy and/or radiation therapy, surgery to remove a tumor, targeted therapy or samarium. Teenagers and children can also enter clinical trials to treat the disease. 

Children and teenagers who endure osteosarcoma treatment may suffer from late effects, such as secondary cancers, learning and memory problems, infertility, organ complications, and more.

Survival rates for osteosarcoma patients vary depending on if the cancer has spread or not. If the cancer has spread beyond the main tumor, a child's 5 year survival rate ranges from 15 to 30 percent, unless it has only spread to the lungs. If it only spread to the lungs, the survival rate is 40 percent. If the cancer stays contained, the 5 year survival rate ranges from 60 to 80 percent. 

The good news is that there is much progress being made for osteosarcoma treatments. A musician by the name of Zach Sobiech and his family, helped to start the Zach Sobiech Osteosarcoma Fund at the Children's Cancer Research Fund. Direct proceeds from his music and various other fundraisers, help scientists and researchers to tackle Osteosarcoma. 

Ewing Sarcoma

Ewing Sarcoma is a cancerous tumor that grows in the bones or in the tissue around the bones - often in the legs, pelvis, ribs, arms, or spine. Ewing Sarcoma can spread to the lungs, bones, and bone marrow. 

Ewing Sarcoma is the second most common type of bone cancer in children, but it is very rare. About 200 children and young adults are found to have Ewing Sarcoma each year in the United States. About half of all Ewing Sarcoma tumors occur in children and young adults between ages 10 and 20, and it affects slightly more boys than girls. 

The symptoms include: swelling and soreness around the tumor area - often commonly mistaken for a sports injury or bumps and bruises that every child gets, a low fever that may seem to be caused by an infection, bone pain - especially pain that worsens during exercise or at night, and limping, which is caused by a tumor on the leg bone. 

Standard treatment for Ewing Sarcoma include: chemotherapy, radiation therapy, surgery, targeted therapy, and high dose chemotherapy with stem cell research. New types of treatment including immunotherapy and a variety of clinical trials, are also surfacing. 

Children and teenagers who undergo treatment for Ewing Sarcoma may suffer from a variety of long lasting effects that affect their organs, growth, development, mental health, and more. They also have a higher risk of developing a secondary cancer. 

About 70% of children with Ewing Sarcoma are cured, however; teenagers ages 15 to 19 have a lower survival rate of approximately 56%. For children diagnosed after their disease has spread, the survival rate lowers significantly, and is less than 30%. Children whose tumors are larger or located in the pelvis, ribs, or spine, are unfortunately, less likely to be cured. 

Rhabdomyosarcoma

Rhabdomyosarcoma is a type of cancerous tumor that arises in the soft tissue, such as the muscles. It can occur throughout childhood and may be present at birth. Rhabdomyosarcoma in children differs from the form of the disease typically seen in adults. There are two types of rhabdomyosarcoma: embryonal and alveolar. 

Embryonal Rhabdomyosarcoma is seen most often in children under 5 years old. This form affects the head and neck, bladder, and sexual organs. 

Alveolar Rhabdomyosarcoma occurs in all age groups and often affects the large muscles of the arms, legs, and trunk. 

Soft tissue sarcomas account for approximately 7 to 8 percent of childhood cancers, with rhabdomyosarcoma being the most common type of soft tissue sarcoma in children. About two thirds of this sarcoma are diagnosed in children younger than 10 years old, and it happens in more males than females. 

The symptoms of Rhabdomyosarcoma are similar to those of other cancers, and may include: a lump or swelling that does not go away, bleeding in the nose, vagina, or rectum, constipation, bulging eyes, and headaches

For treatment, surgery generally occurs first. After surgery, all patients receive chemotherapy. More surgery and/or radiation therapy may be used for some patients. 

Survival rates depend on factors such as tumor size and location, and the amount of tumor that can be removed. More than 70 percent of children with this sarcoma that has not spread survive long term, but sometimes may suffer from lasting effects, such as: infertility, radiated bone or tissue may not grow as well, stiffening of the lungs, poor vision or cataracts, cognitive delays, and more.

Non-Rhabdomyosarcoma

Non-Rhabdomyosarcoma Soft Tissue Sarcomas, or NRSTS, tumors develop in the soft tissues of the body. The most common sites are the arms and legs, but the tumors can occur anywhere. Other sites include, the head and neck region, chest, abdomen, and pelvis. Some types of NRSTS cancers are more likely to develop in children, while some NRSTS tumors may be inherited. 

NRSTS tumors account for about 4% of all childhood cancers. NRSTS is most common in teens and infants younger than 1 year. In people younger than age 20, about 500 to 600 cases are diagnosed each year in the United States.

Often, there are no symptoms for NRSTS. However, an injury or other trauma can make the tumor noticeable. Symptoms may include: pain, swelling, or numbness. Symptoms of advanced NRSTS include: fever, sweating, weight loss, and high blood sugar in children with fibrosarcoma in the lungs.

The most effective NRSTS treatment is surgery to remove the tumor. If surgeons cannot remove the tumor, then radiation and/or chemotherapy may be used to shrink the tumors before they are removed. Patients with tumor remaining after surgery may benefit from radiation therapy and proton therapy. Certain patients with high risk NRSTS may benefit from chemotherapy.

Survival rates for NRSTS vary widely due to the variety and numbers of cancers grouped under this name. Overall, the outlook for survival in patients treated for NRSTS depends on several factors, including: the size of the tumor, its stage of growth and spread, the amount of tumor that can be removed surgically, and the extend of its spread, if any, to other parts of the body.

Other Cancers

Childhood cancers can also be found in the liver, kidneys, and gonads.

Hepatoblastoma

Hepatoblastoma is a type of liver cancer that is usually found in babies and children up to age 3. It is very rare and is diagnosed in fewer than one in a million children. Symptoms of hepatoblastoma include: swollen abdomen, pain the abdomen, back pain, itchy skin, jaundice, pale skin and lips, loss of appetite, weight loss, vomiting, and fever.

Surgery is used to remove all of the tumor, and it is the most important part of treatment. If the entire liver is affected, a liver transplant may be needed to remove all of the tumor. In about half of all children with hepatoblastoma, the tumors are small, and surgeons can remove them right away. If the tumor is large, the patient will usually have chemotherapy before surgery to shrink the tumor and make it easier to remove.

The survival rate is more than 80% if the tumor is only in the liver and can be completely removed with surgery after chemotherapy. Survival rates for children whose tumors involve the entire liver or have spread outside the liver, range from 20% to 70% at three to five years after diagnosis.

Hepatocellular Carcinoma

Hepatocellular Carcinoma is a type of liver cancer that usually affects people whose livers have been under extra strain for a long period of time due to infections, metabolic diseases, or prolonged use of certain drugs. Children are more likely to develop hepatocellular carcinoma in these cases:

- They are infected with Hepatitis B or C

- If they are long time users of androgenic anabolic steroids

- They have congenital diseases and conditions, such as Wilson's Disease, Type 1 Glycogen Storage Disease, etc.

Only about a third of children diagnosed with hepatocellular carcinoma have one of these risk factors.

Hepatocellular Carcinoma is very rare in children, especially those younger than 5. Less than one child in a million is diagnosed with this disease. The disease is usually diagnosed in children between the ages of 12 and 14 with symptoms that include: swelling or pain in the abdomen, a mass or lump in the upper right side of the abdomen, nausea and vomiting, loss of appetite, weight loss, and fever.

Without complete removal of all the tumor with surgery, hepatocellular carcinoma is not curable. This could require a liver transplant if the tumor involves the whole liver. Other treatments may include the following: chemoembolization, chemical injection, radiofrequency ablation, live transplant, or chemotherapy.

The overall survival rate is less than 30% in children, but the patient may become disease free if treated with the complete removal of the tumor, or a liver transplant of the cancer has not spread outside of the liver. Some late effects from treatment and tumor removal include: problems with the heart, kidneys, nerves, and hearing loss.

Kidney Tumors

Wilms Tumor is a solid cancerous tumor of the kidney that arises from immature kidney cells. For most children with Wilms tumor, no clear cause is known, as more research is needed. About 1.5% of children with Wilms tumor have a family member with the disease, and about 5 to 10 percent of children with Wilms tumor have tumors in both kidneys, which may be inherited.

Sometimes, more than one Wilms tumor is present in the affected kidney. This does not mean that the disease has spread. Each of the tumors develops on its own and may come from immature cells within the kidney. If Wilms tumor spreads, it will be partly because of genetics, and partly because of how far the disease has progressed. Wilms tumor may spread into the blood vessels surrounding the kidney as a tumor clot. The other common site for it to spread is the lungs.

Wilms tumor is the 4th most common type of childhood cancer and the most common type of kidney cancer in children. About 500 new cases of Wilms tumor are found each year in the United States, affecting about 1 in every 10,000 children. Children with one kidney affected are usually diagnosed around 3 years of age. Children with bilateral Wilms tumor are usually diagnosed around 2 years of age. Sometimes, Wilms tumor is also found in older children and even adults. 

Wilms tumors can grow quite large before any symptoms develop. When they do appear, symptoms may be similar to those of other diseases. These symptoms include: hardness or swelling in the abdomen that may or may not be painful, fever, nausea, loss of appetite, blood in the urine, constipation, and high blood pressure. 

Treatment involves a combination of surgery and chemotherapy and may also involve radiation therapy for patients with the advanced disease. Long term survival rates are excellent, as 85 to 90% of Wilms tumor with favorable tumor cells can be cured. Cure rates for patients with anaplastic histology, a more aggressive form of Wilms tumor, are lower. Patients who are treated for Wilms tumor should continue close medical follow-up, since chronic health problems occur in 25% of survivors. These health problems are related to high blood pressure, and heart and kidney function. 

Germ Cell Tumors

Germ cells form as an unborn baby develops. These cells are part of a male or female's reproductive system, but sometimes they develop into tumors that are either malignant or benign. Germ cell tumors can develop in the central nervous system, including the brain. They can also develop outside the brain; those tumors are called extracranial and can be grouped into two main types: gonadal or extragonadal.

Extracranial germ cell tumors make up about 3% of all childhood cancers for children under age 15. There are more common in adolescents, ages 15-19, representing 14% of cancers for this age group.

Treatment for this cancer may include surgery and chemotherapy. Radiation may be used as well if the tumor is in the brain. The 5 year survival rate for children with germ cell tumors varies widely based on the type of tumor, location, stage, age of the patient, and more. Overall, germ cell tumors are rare in children, making it difficult for researchers to study, and advance treatment options.

 

No child or parent should ever have to face the horrific diagnosis of cancer. Unfortunately, cancer is a cruel attacker that doesn't discriminate against anyone. With advanced treatment options constantly being formulated, and researchers working around the clock to find a cure, we can all hope that someday, we will see cancer as nothing more than a zodiac sign.