Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version
Unusual Cancers of the Reproductive and Urinary Systems
Bladder Cancer
Bladder cancer is a disease in which malignant (cancer) cells form in the tissues of the bladder. The bladder is a hollow organ in the lower part of the abdomen. It is shaped like a small balloon and has a muscle wall that allows it to get bigger or smaller. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body.
The most common type of bladder cancer is transitional cell cancer. Squamous cell and other more aggressive types of bladder cancer are less common.
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
The risk of bladder cancer is increased in children who have been treated for cancer with certain anticancer drugs, called alkylating agents, which includes cyclophosphamide, ifosfamide, busulfan, and temozolomide.
Bladder cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Blood in the urine (slightly rusty to bright red in color).
- Frequent urination or feeling the need to urinate without being able to do so.
- Pain during urination.
- Abdominal or lower back pain.
Other conditions that are not bladder cancer may cause the same signs and symptoms.
- Physical exam and health history.
- CT scan.
- Ultrasound of the bladder.
- Biopsy.
- Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscopefor signs of cancer. If a cystoscopy is not done at diagnosis, tissue samples are removed and checked for cancer during surgery to remove all or part of the bladder.
See the General Information section for a description of these tests and procedures.
Prognosis
In children, bladder cancer is usually low grade (not likely to spread) and the prognosis is usually excellent after surgery to remove the tumor.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of bladder cancer in children is usually the following:
- Surgery to remove part of the bladder. Transurethral resection (TUR) is a surgical procedure to remove tissue from the bladder using a resectoscope inserted into the bladder through the urethra. A resectoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool to remove tissue and burn away any remaining tumor cells. Tissue samples from the area where the tumor was removed are checked under a microscope for signs of cancer.
- Surgery to remove the bladder (rare).
Talk to your child’s doctor about how this type of surgery can affect urinating, sexual function, and fertility.
Treatment of recurrent bladder cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Bladder Cancer Treatment for more information.
Testicular Cancer
Testicular cancer is a disease in which malignant (cancer) cells form in the tissues of one or both testicles. The testicles are 2 egg-shaped glands located inside the scrotum (a sac of loose skin that lies directly below the penis). The testicles are held within the scrotum by the spermatic cord, which also contains the vas deferens and vessels and nerves of the testicles.
There are two types of testicular tumors:
- Germ cell tumors: Tumors that start in sperm cells in males. Testicular germ cell tumors may be benign (not cancer) or malignant (cancer). The most common testicular germ cell tumors in young boys are benign teratomas and malignant nonseminomas. Seminomas usually occur in young men and are rare in boys. See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on testicular germ cell tumors.
- Non-germ cell tumors: Tumors that begin in the tissues that surround and support the testicles. These tumors may be benign or malignant. Juvenile granulosa cell tumors and Sertoli-Leydig cell tumors are two types of non-germ cell tumors.
Signs and Symptoms and Diagnostic and Staging Tests
Testicular cancer and its spread to other parts of the body may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
A painless lump in the testicles may be a sign of a testicular tumor. Other conditions may also cause a lump in the testicles.
- Physical exam and health history.
- CT scan of the chest, abdomen, or pelvis.
- MRI of the chest, abdomen, or pelvis.
- Ultrasound.
- Biopsy. The tissue removed during surgery is viewed under a microscope by a pathologist to check for signs of cancer.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose testicular tumors include the following:
- Serum tumor marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The tumor marker alpha-fetoprotein is used to diagnose germ cell tumors.
Prognosis
In children, the prognosis is usually excellent after surgery to remove the tumor.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of non-germ cell testicular cancer in children may include the following:
- Surgery to remove the tumor from the testicle.
- Surgery to remove one or both testicles.
Treatment of recurrent non-germ cell testicular cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on testicular germ cell tumors.
Ovarian Cancer
Ovarian cancer is a disease in which malignant (cancer) cells form in the ovary. The ovaries are a pair of organs in the female reproductive system. They are located in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond in an adult woman. The ovaries produce eggs and female hormones (chemicals that control the way certain cells or organs function).
Most ovarian tumors in children are benign (not cancer). They occur most often in females aged 15 to 19 years.
There are several types of malignant (cancer) ovarian tumors:
- Germ cell tumors: Tumors that start in egg cells in females. These are the most common ovarian tumors in girls. (See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on ovarian germ cell tumors.)
- Epithelial tumors: Tumors that start in the tissue covering the ovary. These are the second most common ovarian tumors in girls.
- Stromal tumors: Tumors that begin in stromal cells, which make up tissues that surround and support the ovaries. Juvenile granulosa cell tumors and Sertoli-Leydig cell tumors are two types of stromal tumors.
- Small cell carcinoma of the ovary: Cancer that begins in the ovary and may have spread to the abdomen, pelvis, or other parts of the body. This type of ovarian cancer is fast growing and has a poor prognosis.
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
The risk of ovarian cancer is increased by having one of the following conditions:
- Ollier disease (a disorder that causes abnormal growth of cartilage at the end of long bones).
- Maffucci syndrome (a disorder that causes abnormal growth of cartilage at the end of long bones and of blood vessels in the skin).
- Peutz-Jeghers syndrome (a disorder that causes polyps to form in the intestines and dark spots to form on the mouth and fingers).
- Pleuropulmonary blastoma syndrome (a disorder that may cause cystic nephroma, cysts in the lung, thyroid problems, and other cancers of the kidney, ovary, and soft tissue).
- DICER1 syndrome (a disorder that may cause goiter, polyps in the colon, and tumors of the ovary, cervix, testicle, kidney, brain, eye, and lining of the lung).
Ovarian cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Pain or swelling in the abdomen.
- A lump in the abdomen.
- Constipation.
- Painful or missed menstrual periods.
- Unusual vaginal bleeding.
- Male sex traits, such as body hair or a deep voice.
- Early signs of puberty.
Other conditions that are not ovarian cancer may cause these same signs and symptoms.
- Physical exam and health history.
- CT scan.
- MRI.
- Ultrasound.
- Biopsy. The tissue removed during surgery is viewed under a microscope by a pathologist to check for signs of cancer.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose ovarian tumors include the following:
- Serum tumor marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The tumor markers alpha-fetoprotein, beta-human chorionic gonadotropin (β-hCG), CEA, CA-125, and others are used to diagnose ovarian cancer.
During surgery to remove the tumor, fluid in the abdomen will be checked for signs of cancer.
Prognosis
Ovarian epithelial cancer is usually found at an early stage in children and is easier to treat than in adult patients.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of benign ovarian tumors in children may include the following:
- Surgery.
Treatment of ovarian epithelial cancer in children may include the following:
- Surgery.
- Radiation therapy.
- Chemotherapy.
Treatment of ovarian stromal tumors, including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, in children may include the following:
- Surgery to remove one ovary and one fallopian tube for early cancer.
- Surgery followed by chemotherapy for cancer that is advanced.
- Chemotherapy for cancer that has recurred (come back).
Treatment of small cell carcinoma of the ovary may include the following:
- Surgery followed by chemotherapy and high-dose chemotherapy with stem cell rescue.
- Targeted therapy (tazemetostat).
Treatment of recurrent ovarian cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the following PDQ summaries for more information:
Cervical and Vaginal Cancer
Cervical cancer is a disease in which malignant (cancer) cells form in the cervix. The cervix is the lower, narrow end of the uterus (the hollow, pear-shaped organ where a baby grows). The cervix leads from the uterus to the vagina (birth canal). Vaginal cancer forms in the vagina. The vagina is the canal leading from the cervix to the outside of the body. At birth, a baby passes out of the body through the vagina (also called the birth canal).
The most common sign of cervical and vaginal cancer is bleeding from the vagina. Other conditions may also cause vaginal bleeding. Children are often diagnosed with advanced disease.
Diagnostic and Staging Tests
Tests to diagnose and stage cervical and vaginal cancer may include the following:
- Physical exam and health history.
- Ultrasound.
- MRI.
- CT scan.
- Biopsy. A transvaginal needle biopsy is the removal of tissue using a needle that is guided by ultrasound.
- Bone scan.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose cervical and vaginal tumors include the following:
- Serum tumor marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.
- PAP test: A procedure to collect cells from the surface of the cervix and vagina. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the cervix and vagina. The cells are viewed under a microscope to find out if they are abnormal. This procedure is also called a Pap smear.
- Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
- Proctoscopy: A procedure to look inside the rectum and anus to check for abnormal areas, using a proctoscope. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing the inside of the rectum and anus. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of childhood cervical and vaginal cancer may include the following:
- Surgery to remove as much of the cancer as possible, followed by radiation therapy, if cancer cells remain after surgery or cancer has spread to the lymph nodes.
- Chemotherapy may also be used but it is not yet known how well this treatment works.
Treatment of recurrent cervical and vaginal cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
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