Childhood Cancer

Genitals are human reproductive organs. The female organs are the ovaries, uterus, and vagina. Ovaries, the organs that release eggs and produce sex hormones, are discussed in Chapter 9 . The uterus is an organ with strong, muscular walls. The structure at the bottom of the uterus that connects it to the vagina is the cervix. The vagina is a muscular, tube-shaped organ. It is sometimes called the birth canal because it is the opening through which a baby is born. Figure 14-2 shows the location of female genitals.

Female genitals

Figure 14-2. Female genitals

The testes are the male organs that produce sperm cells and the hormone testosterone. The testes are in a sac called the scrotum, located behind the penis. Each testis is made of coiled tubules called seminiferous tubules. Cells in the tubules produce sperm, which is stored in a structure called the epididymis. The prostate is a walnut-sized gland located just below the bladder. It makes fluid that is mixed with sperm cells to produce semen. The penis is the male organ through which sperm cells leave the body during sexual intercourse. Figure 14-3 shows the location of male genitals.

Male genitals

Figure 14-3. Male genitals

Organ damage in females

Very few girls get tumors in the vagina or uterus. However, some female children or teens with tumors in the pelvic area (i.e., rhabdomyosarcoma, Ewing sarcoma, osteosarcoma) get high-dose radiation that can affect the growth and development of the vagina and/or uterus. Lower doses of radiation, when used with some types of chemotherapy that enhance radiation (e.g., dactinomycin and doxorubicin), can cause the same long-term effects as high-dose radiation alone.

Some female survivors who had abdominal tumors treated with radiation have permanent changes in the size and function of the uterus. These late effects are more likely in girls treated before puberty with more than 2000 cGy of radiation. The uterus of a girl treated after puberty usually is not damaged unless more than 4000 cGy of radiation is used. Radiation can stunt the growth of the uterus and can also cause it to become scarred (fibrotic) and less elastic. This type of damage to the uterus can cause miscarriages of pregnancies or low birthweight children. It occurs most often in survivors of Wilms tumor who had more than 2000 cGy of abdominal radiation and in Hodgkin lymphoma survivors who had radiation to the abdomen and chemotherapy (but not either alone).

I had radiation to my right flank for Wilms when I was 2 years old in 1962. I’ve had three healthy baby boys, all over 7 pounds. The doctors were shocked when they found one of my fallopian tubes was badly damaged from the radiation. It didn’t ever cause me any problems.

Girls or teens who had more than 4000 cGy of radiation to a field that included the vagina can develop fibrosis (when the tissue gets scarred and tough and doesn’t stretch well) and diminished vaginal development. This problem can also occur after lower doses of radiation if radiation-enhancing chemotherapy drugs (e.g., dactinomycin) are also given. Fibrosis can affect the size and flexibility of the vagina, which can alter sexual function and the ability to deliver babies vaginally.

Signs and symptoms in females

Signs and symptoms of uterine damage include the following:

  • Small uterus (your gynecologist will tell you this)

  • Inability to get pregnant

  • Difficulties with menstruation such as irregular periods or heavier than normal flow

  • Miscarriage

  • Low birthweight babies

Signs and symptoms of vaginal damage include the following:

  • Abnormal vaginal bleeding

  • Vaginal dryness

  • Inability to have intercourse due to a small vaginal opening

  • Painful intercourse

Organ damage in males

Males children or teens who had high-dose radiation to the abdomen for rhabdomyosarcoma, Ewing sarcoma, or osteosarcoma are at risk for damage to the prostate gland and the nerves that control sexual functioning. Boys or teens with testicular cancer or relapsed leukemia (in the testes) usually have a testicle removed and the area irradiated.

I had testicular cancer when I was 17, back in 1976. They removed the testicle, then did a retroperitoneal lymph node dissection to check for spread. They did find cancer in two lymph nodes. I had radiation for 6 weeks, then chemotherapy for a year.

Low doses of radiation to the prostate can slow or stop the development of this organ. High doses (more than 5000 cGy) can cause the organ to atrophy (shrink). Because the prostate produces part of the fluid that makes semen, damage to it can reduce or eliminate ejaculation. Nerve damage from surgery or radiation can affect the ability to have an erection and can also affect ejaculation.

Survivors who had radical lymph node dissections (removal of many lymph nodes in the groin) sometimes accumulate excessive fluid in the testicles (called hydrocele). 3 This late effect has been seen in long-term survivors of Hodgkin lymphoma, Wilms tumor, and paratesticular rhabdomyosarcoma.

Signs and symptoms in males

Signs and symptoms of prostate damage include the following:

  • Decreased volume of seminal fluid (ejaculate)

  • Small or atrophied prostate (your healthcare provider will tell you this)

Signs and symptoms of damage to nerves that control sexual function include the following:

  • Inability to have an erection

  • Inability to maintain an erection

  • Inability to have an orgasm

  • Having orgasms without ejaculation

Screening and detection for males and females

Frank discussions with your healthcare provider about changes in your sexual organs or sexual function are essential to identify and treat these late effects. Many people (including some healthcare providers) feel uncomfortable talking about sex and sexuality. However, sexuality is a vital part of your life that influences your sense of self and quality of life. Find a healthcare provider you trust who is comfortable discussing these issues so you can explore all of your options to address late effects involving your genitals.

The radical lymph node dissection I had to see if my testicular cancer had spread damaged the sympathetic nerve that controls ejaculation. I’m normal sexually in every way except I can’t ejaculate. This side effect was not explained well by the surgeon at the time. There I was, 17 and sitting in the room with my parents, when he said, “You might have problems with ejaculation.” That’s all. I didn’t have a clue what he was talking about. It really worried me at the time. I thought I might never have an erection again. Twenty-two years later, I realize that the big loss is fertility. I still struggle with that.

Unfortunately, doctors are very uncomfortable talking about this type of late effect. No one asks me about erections, ejaculation, or the problems they cause. These things have changed my life in a big way and I have no one to talk to about it.

Healthcare providers for males should take an age-appropriate history that focuses on any problems with libido (sex drive), sexual function, or fertility. For males, the prostate gland is felt manually, and sometimes an ultrasound of the organ is done to evaluate size. Late effects involving the testicles are covered in Chapter 9 .

Healthcare providers for female survivors should take an age-appropriate history that focuses on any problems with libido, sexual function, or fertility. Female children and teens need regular evaluation of their sexual development to ensure that puberty is proceeding normally. Women and sexually active teens should have a yearly pelvic examination. This exam may need to be done under anesthesia or sedation for women or teens who have small vaginas or vaginal fibrosis. The uterus can be evaluated using ultrasound, computed tomography (CT) scan, or magnetic resonance imaging (MRI). Abdominal radiation that included the ovaries requires extensive evaluation, outlined in Chapter 9 .

A nurse practitioner who cares for survivors of childhood cancer said:

I have talked to many survivors with problems with sexual performance. Teenagers come in after becoming sexually active with both physical and psychological problems about sex. Many adults have lived for years with late effects that impact their ability to have satisfying sex. I talk to them openly and honestly about the issues. I ask them candid questions about sexual functioning, which include their satisfaction with their sex life, performance issues, and libido.

Sometimes we find a physical cause for sexual difficulties such as low testosterone or estrogen. Other times it involves their feelings about their appearance or it reflects problems in the relationship. Whatever the cause, it is our job as healthcare providers to initiate the conversation and help make them feel comfortable sharing these intimate details of their lives. That enables us to get them the help that they need and deserve.

Medical management for females and males

Female teens and women with late effects that alter sexual functioning or fertility need to be referred to a gynecologist and/or endocrinologist for further evaluation, testing, and treatment. Women or female teens with vaginal late effects may need vaginal dilations or reconstructive surgery. Survivors should consult a gynecologist with extensive experience doing these procedures. A woman with a small uterus or who has uterine fibrosis needs counseling about pregnancy. Pregnant women who had pelvic radiation should get their prenatal care from an obstetrician who specializes in high-risk pregnancies.

At the age of 7, I was diagnosed with AML. After countless rounds of chemotherapy followed by total body radiation, I received a life-saving bone marrow transplant from my older brother, Nathan. As a result of the intensive cancer treatment, I experience chronic health issues such as a cardiomyopathy, endocrine dysfunction, and cataracts in both eyes. When I was 24, I was diagnosed with a secondary cancer—papillary thyroid cancer—a result of the total body radiation I received.

Due to the total body radiation and the cyclophosphamide that I received as a child, I was told that I would be sterile. Coming from a large family, I had always dreamed of having a child of my own. On April Fool’s Day, when I was 28 years old, I was taking a routine pregnancy test prior to a medical procedure to scan for thyroid cancer and discovered I was pregnant! Twelve weeks pregnant!

I was fortunate to have both a fabulous oncologist and OB/GYN who managed my high-risk pregnancy. My cardiomyopathy was exacerbated by the pregnancy, leading to increased hypertension, which was controlled by beta blockers. The total body radiation I received led to impaired uterine growth and a shortened cervix. Despite a cervical cerclage being placed in addition to being on bed rest, at 25 weeks gestation my daughter—Hope Isabella—arrived weighing 1 pound 7 ounces.

After 111 days in the NICU (neonatal intensive care unit) fighting for her life, she came home and continues to grow strong. To me, Hope is the epitome of a medical miracle. She is a wonderful example of how far we have come scientifically. She was born from an early BMT survivor who wasn’t supposed to live to see her 8th birthday, and who was certainly not expected to be able to bear children. But, she also represents how far we have left to go to maintain cure rates while reducing late effects of treatments.

Male teens or men with late effects that alter sexual functioning or fertility need to be referred to a urologist and/or endocrinologist for further evaluation, testing, and treatment. Males who had a testicle removed may want to discuss having a prosthesis implanted. Those who develop a hydrocele usually have the fluid surgically drained.

I had my urine tested to see if I had retrograde ejaculation (ejaculate backing up into the bladder), but there were no sperm there. The only way to see if I’m fertile is to biopsy the remaining testicle. Since there is a small chance of damaging the testicle, I haven’t had that done. The technology has improved so much that they say they only need a few sperm to fertilize an egg. But each time would cost $10,000 and would only have a 30 percent chance of success. I wish I was just back to the way I was before.

• • • • •

When the doctors told me that I probably was infertile, it was the most devastating part of the whole experience. By the time they told me about sperm banking it was too late. My urologist looked at the results and said, “No way, buddy.” When I was dating, I told my future wife that I was told I’d be infertile. But in the back of my mind, I always had hope. My wife stayed on the pill until we thought we were ready to be parents. The first month she went off, she got pregnant. We have an 18-month-old boy, and my wife is pregnant again. It’s wonderful. He’s a miracle.

Any survivor with sexual problems that result from treatment for childhood cancer needs both medical and psychological follow-up. A team approach that provides psychological help to address concerns about body image, fertility, or sexuality is crucial.

I’ve never told any of my girlfriends what type of cancer I had. I have huge abdominal scars so I have to say something. I usually tell them I had intestinal cancer. I still struggle about the infertility. I’d love to have a family, but just can’t discuss it with girlfriends. As soon as we start getting serious, girlfriends usually bring up kids, and I just don’t want to go there. I can’t seem to find a way to have that conversation.

Centers with comprehensive long-term follow-up programs have multidisciplinary teams that include psychologists and social workers who are familiar with survivors’ sexual issues and concerns. They can provide information, support, and one-on-one assistance with how to address these issues in relationships.