Childhood Cancer

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Childhood Cancer Survivors

Genetic Predisposition

Cancers with known genetic (hereditary) causes sometimes carry a greater risk of second cancers. For instance, survivors of the genetic form of retinoblastoma and those with Li Fraumeni Syndrome should be evaluated regularly for the rest of their lives, because they have a much higher chance of developing second tumors than do other survivors. See Chapter 6, Genetic Testing and Childhood Cancer for more information on the cancer predisposition syndromes (CPS) and why some children may be more at risk for cancers. One important thing to remember is that CPS place some children at higher risk for developing a primary (first new) cancer and not necessarily a second cancer. More information is being discovered about the genetics of cancer. However, there is more we need to learn about the relationship between chemotherapy, radiation and the development of second cancers in children with CPS.

Some types of cancer (colon, breast, and ovarian) run in families (hereditary), so it is important to discuss your family history with your healthcare provider. Speaking with a genetic counselor to discuss your personal or family history of cancer can help you to understand your risks of developing cancer. Genetic counselors can help with a risk assessment and walk you through genetic testing options. Knowing your risk factors can be helpful in determining your medical management moving forward. It is important to know that if you have a genetic mutation in a cancer predisposition gene, this does not mean you will develop cancer again. Survivors with many risk factors may live long and healthy lives and never get cancer again, while some people at low risk will get cancer. The facts your healthcare provider discusses with you are probabilities for large groups of people; no one can predict what will happen to you.

I have a family history of brain cancer, breast cancer, and thyroid issues. My doctors are aware of these and do checks when I go in for routine doctor’s appointments. They also make me aware of any warning signs that I should be aware of so that I can schedule follow-up appointments if need be. I see my doctors regularly and am honest with them about my symptoms/how I feel. I am also very in tune with my body, and I just know when something feels off or wrong or unusual. When something concerns me, I go to the doctor, as I’d rather know that it’s nothing to worry about than just worry about it on my own.

 

After chemotherapy, I had to do 14 rounds of proton radiation every day. I was extremely scared about this because of where my cancer was located. I had tumors in my chest and neck that needed radiation. My doctors warned me about the chance of developing breast cancer, heart disease and lung damage from the radiation. This has added to my anxiety. I have tried to be super proactive about following up with my doctors since ending radiation. I make sure I keep up with doctor appointments, scans, and annual exams to ensure the best chance possible of never getting any other sickness or disease. Although nothing can be promised, I feel better knowing I have surveillance of so many aspects of my health.

Advocating for myself has been one of the biggest things I have learned. It’s so important to go to the doctors and get checked! You know your body best, and if something feels wrong or off, just get it checked, because you never know. It’s better to be safe than sorry. Advocating for yourself is one of the best things you can do for yourself.

 

My daughter was diagnosed with neuroblastoma at 2 ½ months. When she was 7 1/2, she was diagnosed with a secondary cancer, renal cell carcinoma. Doctors described it as a secondary cancer because it was caused by chemotherapy treatment from the first cancer. This was rare because renal cell carcinoma is not usually found in young children as a secondary cancer.

 

The risk of developing a secondary cancer sometimes scares me, but then I tell myself, let’s go for round two -- I’m not scared; if I did it once, I’ll do it again. I do everything I can to take care of my body and monitor myself thoroughly for any signs of secondary cancer.

 

I asked the doctor about potentially having a child with cancer before we started trying. They told me it was not a genetic illness, so that helped calm my fear. I worry about my son’s health constantly. I am always wondering where a bruise came from or if he’s eating enough. I think all moms are like that, though. I just am very extreme when it comes to worrying!

Risk of treatment

Survivors at highest risk for second cancers are those who:

  • Were treated with radiation

  • Received the following agents: classical alkylating agents (busulfan, carmustine, chlorambucil, cyclophosphamide (Cytoxan®), ifosfamide, lomustine, mechlorethamine, melphalan, procarbazine, thiotepa, heavymetals, carboplatin, cisplatin); Non-classical alkylators: (dacarbazine, temozolomide); anthracyclines (daunorubicin, doxorubicin, epirubicin, idarubicin, mitoxantrone) (COG V 6.0 section 16). Any of these medications can cause a secondary cancer, but higher doses of any or combinations of these drugs can increase the risk for second cancers.

  • Have genetic diseases that carry an increased risk

  • Are included in Section 7 of the COG Survivorship Guidelines (v6.0)

The screening allows for earlier detection and treatment should you develop another cancer, increasing your chance for early treatment and cure. It also gives you the opportunity to discuss ways of lowering your risks by making healthy lifestyle and behavior choices.

Radiation and some types of chemotherapy can increase the risk of second cancers. Combined radiotherapy and chemotherapy may play an additive role in the development of second cancers (Dracham CB, et al., 2018). Some types of second cancer are very easy to cure such as skin cancers and thyroid cancers.

Radiation

In general, higher doses of any radiation increase the risk of developing a second cancer. More recent protocols have used lower radiation doses (in some cases), more precise techniques, and radiation given in smaller fractions, allowing the healthy tissue to repair itself (COG V 6.0). It is hoped that the use of the new proton-beam radiation therapy will significantly decrease the risk of damage to healthy tissue. However, we do not yet know the long-term outcomes and whether the risk for secondary cancer will be significantly reduced in survivors who receive proton therapy (Indelicato DJ, et al. 2021).

Radiation kills cancer cells and may also cause changes in normal cells that are exposed to the radiation. In some cases, cancers can develop in the irradiated areas. For instance, female survivors of Hodgkin lymphoma (formerly called Hodgkin’s disease) treated in the 1970s and 1980s who had more than 3600 centigrays (cGy) of mantle (chest) radiation have increased risk of developing breast cancer, often at an early age. Scatter radiation (radiation that escapes into areas around the tumor site) is less common now that radiation techniques have improved.

Thyroid tumors are common tumors that can develop following radiation delivered to the cranial/spinal region, head and neck, chest, or total body radiation given before a stem cell transplant. Younger children at time of treatment are at greater risk for thyroid tumors. Thyroid tumors can be either benign or malignant, and the malignant tumors are very treatable.

Radiation to the pelvis or abdomen (in higher doses) is associated with an increased risk of colon cancer. The current recommendation for follow up is to have a colonoscopy at age 35 or 10 years after the radiation, whichever occurs last.

Chemotherapy

Several chemotherapy drugs as listed above are associated with second cancers in some survivors. Examples include:

  • Alkylating agents: procarbazine, nitrogen mustard, cyclophosphamide (Cytoxan®), ifosfamide, melphalan, and nitrosoureas. High doses of these drugs can cause myelodysplastic syndromes (bone marrow abnormalities that are similar to leukemia) as well as AML.

  • Epipodophyllotoxins: VP-16 (etoposide), VM-26 (teniposide) (Smith MA, et al. 1999).

  • Platinum analogs: cisplatin, carboplatin. Research has not clarified the risk of cancers after treatment with platinum analogs such as cisplatin or carboplatin. Most AML or myelodysplastic syndromes occur when these drugs are given in conjunction with alkylating agents or epipodophyllotoxins.

  • Anthracycline: daunorubicin, doxorubicin, epirubicin, idarubicin, and mitoxantrone may be associated with the development of second acute myeloid leukemia.

  • Targeted therapies: With the addition of precision medicine and testing for targeted genetic variants to help in the management of children with cancer, new agents (drugs) are being used and developed to treat the child’s cancer. We do not have long-term outcomes on the Tyrosine Kinese Inhibitors (TKIS), CAR-T therapy, and other targeted medications and the long-term effects on childhood cancer survivors. Please discuss any long-term effects of these newer drugs/treatment with your provider. Each survivor is different, and long-term follow-up is important.

Children and adolescents who were treated with an immunosuppressant (e.g., cyclosporine or FK 506) have a small chance of developing disorders of the lymph system, including lymphoma. These disorders sometimes resolve without treatment when the immune system is no longer suppressed.

The bladder issue proved to be another blessing-in-disguise and led the doctors to discover her secondary cancer when she was seven and a half. The bladder defect caused her to have constant urinary tract infections (UTls). It was while treating her for the UTIs that they discovered the solid mass which turned out to be Stage I renal cell carcinoma in her left kidney.

I tell everyone that our daughter is a survivor and she is here for a purpose. Her treatments for both cancers were quick and successful, so I just try to give everyone hope. Thankfully, she was fortunate enough to not have any late side-effects. So, I can truthfully tell others that she had a positive outcome from her treatments.

Follow-up exams, early detection and cancer prevention

Once you know what your risk might be, what do you do then? Practical ways to deal with this potentially upsetting information are to take precautions to prevent a second cancer and know the warning signs and attend follow-up survivor clinic visits every year. Notify your healthcare provider immediately if you have any changes in your health status or concerns.

Although you cannot change a genetic predisposition or undo the damage from radiation or chemotherapy, you can decrease your risk factors by making healthy lifestyle choices to reduce your risk. There are numerous ways to prevent cancer outlined in Chapter 5, Staying Healthy.

There are actions you can take to help control the risk factors for developing second cancer, for example, making sure you have periodic healthcare check-ups and making healthy lifestyle choices. Your healthcare provider should perform a thorough assessment each year and discuss any risks you have. The factors that determine which specific tests you need each year depend on the specific treatments you received for childhood cancer, past or current behaviors that affect your risk, and your personal or family history of disease.

Your follow-up examinations should include updating your family medical history with special attention to cancers that have developed in first-degree relatives (your mother, father, brothers, sisters, and children). This valuable information can be missed if family medical history is not updated at regular intervals.

All areas that were irradiated should be visually inspected. Areas where scatter radiation (radiation that escapes into areas around the tumor site) may have occurred also need to be examined. The necks of survivors who receive any head and neck radiation should be palpated (felt by hand) yearly. The results of a thorough physical evaluation and preliminary screening determine the need for additional tests. It’s very important that female survivors who received chest, axilla, and or total body radiation get frequent follow-up care that includes annual mammograms starting at age 25, or 8 years after radiation, whichever comes later (Mulder et al., 2020). They also need to be taught breast self-examination because they can develop breast cancer at an early age.

You can help control some risk factors for developing second cancer, for example, yearly healthcare check-ups and follow-up screenings. Make healthy lifestyle choices and take preventive measures such as performing body self-checks, using sunscreen, eating healthy, and getting exercise.

Know your own body, and if you have any changes/concerns, check with your healthcare provider. Routine yearly examinations will not detect all subsequent cancers. Healthcare providers rely on you to bring concerns or abnormalities immediately to their attention. Try to feel confident in your ability to notice if something in your body does not feel right. If you have an ache or a pain that does not go away, it should probably be checked out. If you notice a lump in the area of your body that was irradiated, go immediately to have it evaluated. It probably will not be cancer, but having it evaluated will provide either reassurance or an early detection of a problem—both good outcomes.

Steps you can take for prevention and early detection/treatment

  • Making healthy lifestyle choices (See Chapter 5, Staying Healthy)

  • Yearly check-ups at your survivors’ clinic or long-term follow-up clinic

  • Periodically perform body self-checks for skin cancers or lumps and have these checked by your healthcare provider. If you had retinoblastoma and radiation treatment to the orbit, regularly examine the socket yourself for any changes. By examining the orbit regularly, you are taking good care of yourself and making an investment in your future.

  • Routinely use sunscreen of at least 30 SPF when you are going to be exposed to sun for long periods of time and try to avoid prolonged direct sun exposure during hours of 10 to 4 pm

  • Check-in with your healthcare provider if you have any concerns

Take care of your emotional/mental health

Another way to think of this issue is to try to find a place in your life to remember your cancer history without letting it control your life or future plans. Don’t trivialize or dismiss what’s happened to you and your family—it was a significant part of your life and shaped your interests and character. And yet, your cancer history is only one part of who you are, and shouldn’t determine your future life, possibilities, and happiness. (For detailed discussion, See Chapters 2 Emotions and Chapter 3, Relationships)

Risk Of Getting A Second CancerReferences

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