Unusual Cancers (Childhood)


General Information

The tumors discussed in this summary are many and different, and the discussion is arranged in descending order from tumors of the head and neck to tumors of the urinary system and skin. All of these cancers are rare enough that most pediatric hospitals might see fewer than two cases in a year. Most of these tumors are more frequent in adults with cancer; thus, much of the information about these tumors may also be sought through sources relevant to adults with these tumors.

Head and Neck Cancers

Head and neck cancers include cancers of the nose and throat, thyroid tumors, mouth cancer, salivary gland cancer, and cancer of the larynx (or voice box). These cancers are discussed below.

Cancer of the Nose and Throat

Cancers that start in the lining of the nasal cavity and throat are called nasopharyngeal cancers. The incidence of this tumor is approximately 1 in 100,000 persons younger than 20 years in the United States.

Nasopharyngeal cancer occurs in association with Epstein-Barr virus (EBV) infection, the virus associated with infectious mononucleosis. This cancer most frequently spreads to lymph nodes in the neck, which may alert the patient, parent, or physician to the presence of this tumor. The tumor may spread to the nose, mouth, and pharynx, causing snoring, nosebleeds, obstruction of the Eustachian tubes, or hearing loss. It may invade the base of the skull, causing cranial nerve palsy or difficulty with movements of the jaw (trismus). The cancer may spread to distant sites such as the bones, lungs, and liver.

Treatment combines the use of surgery, radiation therapy, and chemotherapy. Nasopharyngeal cancer generally has spread to bones of the skull and to lymph nodes in the neck at the time of diagnosis; thus, the principal role of surgery is to obtain adequate diagnostic material from a biopsy of the involved lymph node or the primary site.

Thyroid Tumors

Tumors of the thyroid (a gland near the windpipe that produces thyroid hormone, which helps regulate growth and metabolism) are classified as adenomas or carcinomas. Adenomas are benign (noncancerous) growths that may cause enlargement of all or part of the gland, which extends to both sides of the neck and can be quite large. Some of these tumors may secrete hormones. Transformation to a malignant carcinoma (cancer) may occur in some cells, which then may grow and spread to lymph nodes in the neck or to the lungs.

Most thyroid carcinomas occur in girls. Thyroid carcinomas are differentiated tumors, meaning that they tend to grow slowly and are not highly malignant.

Surgery is the treatment required for all thyroid tumors. A total thyroidectomy (complete surgical removal of the thyroid) or removal of a portion of the thyroid is recommended, depending on the type of tumor. After surgery, hormone replacement therapy must be given to compensate for the lost thyroid hormone. Evaluations at intervals of 4 to 6 months are required to determine whether the cancer has spread to the lungs. Patients with thyroid cancer generally have an excellent survival with relatively few side effects. Even patients with tumor that has spread to the lungs may expect no decrease in life span after appropriate treatment. (Refer to Adult Thyroid Cancer Treatment for more information.)

Mouth Cancers

Cancer of the mouth in children or in adolescents is extremely rare. Mouth cancer primarily occurs in adults older than 50 years who have used tobacco for many years; however, it can occur in people who have had other childhood tumors and had radiation therapy to this area. Evidence suggests that mouth cancer at younger ages primarily results from the use of smokeless tobacco products among preadolescent boys. Changes in the texture, color, and shape of tissue inside the mouth have been seen in more than half of all teenagers who use smokeless tobacco. Precancerous lesions are common among children. Squamous cell carcinoma, the most frequent type of cancer in these areas, must be distinguished from benign (noncancerous) tumors of the throat and neck. Other tumors in this area may include ameloblastoma or adamantinoma, rare tumors that may arise in the bones of the jaw. (Refer to Adult Oropharyngeal Cancer Treatment for more information).

Salivary Gland Cancers

Salivary glands are the parts of the mouth and throat that produce saliva. Many of the tumors in these areas arise in the parotid gland. About 15% of these tumors may arise in the submandibular glands or in the minor salivary glands under the tongue and jaw. These tumors are most frequently noncancerous but on very rare occasions may be malignant (cancerous). The malignant lesions include adenocarcinoma, undifferentiated carcinoma, acinic cell carcinomas, and mucoepidermoid carcinoma. These tumors may occur after radiation therapy for treatment of primary leukemia or solid tumors. Complete surgical removal is the treatment of choice whenever possible, with additional use of radiation therapy and chemotherapy. Prognosis (outcome) for patients with these tumors is generally good. (Refer to Adult Salivary Gland Cancer Treatment for more information.)

Cancer of the Larynx

Benign and especially malignant (cancerous) tumors of the larynx (voice box) are rare. Malignant tumors may be associated with benign tumors such as polyps and papillomas. These tumors may cause hoarseness, difficulty swallowing, and enlargement of the lymph nodes of the neck. Rhabdomyosarcoma (a malignant tumor of muscle tissue) is the most common malignant tumor of the larynx in the pediatric age group. Squamous cell carcinoma of the larynx should be managed with surgery and radiation. Laser surgery may be the first type of treatment used for these cancers.

Papillomatosis of the larynx is a benign overgrowth of tissues lining the larynx. This condition is not cancerous. These tumors can cause hoarseness because of their association with wart-like nodules on the vocal cords; they may extend into the lung and develop into cancer in the larynx. (Refer to Adult Laryngeal Cancer Treatment for more information.)

Thoracic Cancers

Thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid tumors, pleuropulmonary blastoma, esophageal tumors, thymomas, tumors of the heart, and mesothelioma.

These thoracic cancers are discussed below:

Breast Cancer

Most tumors that involve the breast during childhood are benign (noncancerous), yet cancers have been reported in both males and females younger than 21 years. There is an increased lifetime risk of breast cancer in female survivors of Hodgkin's disease who were treated with radiation to the chest area. Mammograms should start at age 25 for any patient who has had radiation therapy to the chest. Treatment options include radiation, chemotherapy, and surgery for children and adolescents with breast cancer. Breast tumors may also occur as cancers that have spread from other types of cancer such as leukemia, rhabdomyosarcoma, or other sarcomas. (Refer to Adult Breast Cancer Treatment for more information.)

Bronchial Adenomas/Carcinoids

Bronchial adenomas (tumors in the trachea or large bronchi, the large airways of the lung), which are slow-growing cancers, are also called carcinoid tumors. Primary treatment of these tumors is surgery to remove the tumor. For bronchial carcinoid tumors, neither chemotherapy nor radiation therapy is indicated, unless evidence of metastasis (spread of cancer to other areas of the body) is documented.

Pleuropulmonary Blastoma

Pleuropulmonary blastomas are rare tumors that usually occur under the tissue covering the lungs. The tumors may recur or spread, in spite of surgical removal. Responses to chemotherapy have been reported. Radiation may be used when the tumor cannot be surgically removed. A family history of cancer in close relatives has been noted for many young patients affected by this tumor.

Esophageal Tumors

Cancer of the esophagus (the muscular tube through which food passes from the throat to the stomach) is rare in the pediatric age group. Symptoms are related to difficulty in swallowing, and associated weight loss. Treatment options for esophageal cancer include either radiation therapy or chemotherapy. Prognosis generally is poor for this cancer, which rarely can be completely removed by surgery. (Refer to Adult Esophageal Cancer Treatment for more information.)

Thymomas

A cancer of the thymus (an organ in the chest, behind the breastbone) is not considered a thymoma (cancer) unless there are cancerous changes of the epithelial cells that cover the organ. Other tumors that involve the thymus gland include lymphoma (cancer that arises in cells of the lymphatic system) and germ cell tumors (tumors that begin in cells that give rise to sperm or eggs); these tumors must be differentiated from true thymomas.

Thymomas are rare in adults as well as children. Various diseases and syndromes are associated with thymomas, including myasthenia gravis, polymyositis, systemic lupus erythematosus, rheumatoid arthritis, thyroiditis, and pure red cell aplasia. Endocrine (hormonal) disorders such as hyperthyroidism, Addison's disease, and panhypopituitarism can also be associated with a diagnosis of thymoma.

Thymomas are usually located in the front part of the chest and are usually discovered during a routine chest x-ray. Symptoms can include cough, difficulty with swallowing, tightness of the chest, chest pain, and shortness of breath, although nonspecific symptoms may occur. These tumors generally are slow growing but are potentially invasive, with cancer spreading to distant organs or lymph nodes. Surgery is performed with the goal of a complete removal. Radiation therapy is necessary for patients with invasive thymoma, whether or not there has been surgery. Chemotherapy is usually reserved for patients with advanced-stage disease who have not responded to radiation therapy or steroids. The prognosis for patients with invasive thymoma usually is poor, although significantly higher survival rates have been reported for patients with tumors that have not spread to the surrounding areas. (Refer to Adult Malignant Thymoma Treatment for more information.)

Tumors of the Heart

Primary tumors of the heart may include benign (noncancerous) and malignant (cancerous) teratoma (a tumor made up of a mixture of tissues), rhabdomyosarcoma (a tumor of muscle tissue), hemangioma (a usually benign tumor made up of blood vessels), and chondrosarcoma (a type of cancer that forms in cartilage). Symptoms include abnormalities of heart rhythm, enlargement of the heart, fluid in the pericardial sac, and congestive heart failure. Successful treatment requires surgery (which may include transplantation) and chemotherapy appropriate for the type of cancer that is present.

Mesothelioma

Mesothelioma can involve the tissue coverings of the lung, the heart, or the abdominal organs. These tumors can spread over the surface of organs, without invading far into the underlying tissue, and may spread to nearby or distant lymph nodes. Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation. In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation. The amount of exposure required to develop cancer is unknown, and there is no information about the risk of children exposed to asbestos. (Refer to Adult Malignant Mesothelioma Treatment for more information.)

Abdominal Cancers

Abdominal cancers include cancer of the adrenal cortex, kidney cancer, stomach cancer, cancer of the pancreas, colorectal cancer, carcinoid tumors of the lung or intestine, and multiple endocrine neoplasia syndrome.

These abdominal cancers are discussed below:

Cancer of the Adrenal Cortex

The adrenal cortex is the outside layer of the adrenal glands. The adrenal glands are a pair of organs near the front side edge of the kidney; their function is to produce hormones such as glucocorticoid and epinephrine. Cancers in this area are classified as carcinomas and adenomas. Adenomas are generally benign, whereas adrenocortical carcinomas frequently secrete hormones and may cause the patient to develop masculine traits, regardless of the patient's gender. Pediatric patients with adrenocortical carcinoma often have Li-Fraumeni syndrome, an inherited condition that predisposes family members to multiple cancers, including breast cancer, rhabdomyosarcoma, and osteosarcoma (cancer of the bone).

These tumors can involve the kidneys, lungs, and bones. Surgical removal should be attempted but may not always be possible if the tumor has spread widely. Additional treatment may include the use of an artificial hormone that blocks the masculinizing effects of the tumor. The prognosis is generally excellent for patients who have small tumors that have been completely removed by surgery, but prognosis can be poor for patients who have large primary tumors or metastatic disease (disease that has spread to other parts of the body) at diagnosis. (Refer to Adult Adrenocortical Carcinoma Treatment for more information.)

Kidney Cancer

Renal cell carcinoma (cancer of the kidney) occurs rarely in children. The annual incidence rate is approximately 4 cases per 2 million children. Renal cell carcinoma may be associated with von Hippel-Landau disease, a hereditary condition. Renal cell carcinoma has also been associated with tuberous sclerosis, a hereditary disease characterized by benign (noncancerous) fatty cysts in the kidney. Renal cell carcinoma usually presents as an abdominal mass, and there may be discomfort, pain, or blood in the urine. The tumor can spread to the lungs, bones, liver, and lymph nodes and often has spread before the diagnosis is made. The primary treatment includes total surgical removal of the kidney and associated lymph nodes. Consideration should also be given to treatment with radiation therapy, chemotherapy, or both. Treatment of metastatic disease (cancer in other parts of the body) is presently unsatisfactory but usually includes the use of immune system modulators such as interferon-alfa and interleukin-2. Rare spontaneous disappearance of lung metastasis may occur with removal of the primary tumor. (Refer to Adult Renal Cell Cancer Treatment for more information.)

Cancer of the Stomach

The frequency of, and death rate from, stomach cancer has declined worldwide over the past 50 years with the introduction of food preservation practices such as refrigeration. Symptoms of stomach cancer include vague upper abdominal pain, which can be associated with poor appetite, and weight loss. Many individuals become anemic but otherwise show no symptoms before the development of metastatic spread. Other symptoms may include nausea, vomiting, change in bowel habits, poor appetite and weakness, and Helicobacter pylori infection.

Treatment should include surgery. For individuals who cannot have a complete surgical removal of tissue, radiation therapy may be used along with chemotherapy. Prognosis depends on the extent of the disease at the time of diagnosis and the success of treatment that is appropriate for the clinical situation. Because of the rarity of stomach cancer in the pediatric age group, little information exists regarding treatment outcomes of children. (Refer to Adult Gastric Cancer Treatment for more information.)

Cancer of the Pancreas

Tumors of the pancreas (a gland in the abdomen that makes pancreatic juices and produces hormones) are rare in children and adolescents. Tumors included within the general category can arise at any site in the pancreas. Most pancreatic tumors do not secrete hormones, although some tumors secrete insulin, which can lead to symptoms of weakness, fatigue, hypoglycemia, and coma. If a tumor interferes with the normal function of the islet cells (cells in the pancreas that produce hormones), patients may have watery diarrhea or abnormalities of salt balance. At times, there is obstruction of the head of the pancreas, which is associated with jaundice and gastrointestinal bleeding.

Treatment includes various surgical procedures to remove the pancreas and duodenum or part of the pancreas. For pediatric patients, the effectiveness of radiation therapy is not known. Chemotherapy may be useful for treatment of localized or metastatic pancreatic carcinoma, although few cases have been successfully treated. Response rates and survival rates generally are not good. (Refer to Adult Pancreatic Cancer Treatment for more information.)

Colorectal Cancer

Cancer of the large bowel is rare in the pediatric age group: 1 person per 1 million younger than 20 years in the United States annually. These tumors can occur anywhere in the colon or rectum and are often associated with a family cancer syndrome. There is an increasing risk of colorectal cancer in members of families with a family history of intestinal polyps, which can lead to the development of multiple adenomatous polyps (benign tumors). Juvenile polyps are not associated with an increased incidence or risk of cancer.

Colorectal cancer usually presents with symptoms related to the site of the tumor. Changes in bowel habits are associated with tumors of the rectum or lower colon. Tumors of the right colon may cause more subtle symptoms but are often associated with an abdominal mass, weight loss, decreased appetite, and blood in the stool. Any tumor that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumor cells within the abdominal cavity.

Colorectal carcinoma is rarely diagnosed in a pediatric patient; however, vague gastrointestinal symptoms should alert the physician to investigate this possibility. Most patients present with evidence of metastatic disease (cancer that has spread to other body parts), either as gross tumor or as microscopic deposits in lymph nodes, on the surface of the bowel, or other organs within the abdomen. Complete surgical removal should be the primary aim of the surgeon, but in most instances this is impossible; removal of large portions of tumor provides little benefit for the individuals with extensive metastatic disease. Most patients with microscopic metastatic disease generally develop gross metastatic disease, and few individuals with metastatic disease at diagnosis become long-term survivors.

Current therapy includes the use of radiation therapy for rectal and lower colon tumors, in conjunction with chemotherapy. (Refer to Adult Colon and Rectal Cancer Treatment for more information.)

Carcinoid Tumors

Carcinoid tumors can involve the lining of the lung or the large or small bowel and may not be cancer. Most lung lesions are not cancerous. Treatment of metastatic carcinoid tumors of the large bowel or stomach becomes more complicated and requires treatment similar to that given for colorectal cancer.

Genital/Urinary Tumors

Genital/urinary tumors include bladder cancer and ovarian cancer.

These cancers are discussed below:

Bladder Cancer

Bladder cancer is extremely rare in children. The most common carcinoma to involve the bladder is transitional cell carcinoma, which generally presents with blood in the urine. The diagnosis and treatment of bladder cancer are the same for children, adolescents, and adults. Adolescents who develop this tumor are often prone to the development of other cancers. Bladder cancer in adolescents may develop as a consequence of certain chemotherapy drugs given for other childhood tumors or leukemia. (Refer to Adult Bladder Cancer Treatment for more information.)

Ovarian Cancer

Most cancers that affect the ovaries are of germ cell origin (beginning in cells that give rise to sperm or eggs), which are more common in children than adults. Treatment is stage related and may include radiation as well as chemotherapy. (Refer to Adult Ovarian Cancer Treatment for more information.)

Other Rare Childhood Cancers

Other rare childhood cancers include skin cancer, clear cell sarcoma of tendon sheaths, and cancer of unknown primary site.

These other rare childhood cancers are discussed below:

Multiple Endocrine Neoplasia Syndrome

Multiple endocrine neoplasia (abnormal and uncontrolled cell growth) syndromes are familial disorders that include cancerous changes in more than 1 endocrine organ at the same time (endocrine tissue secretes hormones). These changes may include hyperplasia (overgrowth of tissue) or benign (noncancerous) tumors. The distinct involvement of multiple glandular structures are referred to as MEN-1 (Werner's syndrome) which may involve tumors of the pituitary gland and parathyroid, adrenal, gastric, and pancreatic structures. MEN-2a (Sipple syndrome) is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as pheochromocytoma; MEN-2b is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as pheochromocytoma, mucosal neuromas, and ganglioneuromas. An additional complex is referred to as the Carney complex, which is associated with heart and skin tumors.

Patients with the MEN-2b syndrome may have a slender body build, long and thin extremities, a high arch palate, and pectus excavatum ("funnel chest") or pes cavus ( a foot deformity characterized by an abnormally high arch). The face may be characterized by thick lips because of mucosal neuromas. In this syndrome, medullary thyroid cancer may be particularly aggressive; therefore, the thyroid should be removed by age 5 or 6 years in affected individuals.

The outcome for patients with the MEN-1 syndrome is generally good, provided adequate treatment can be obtained for parathyroid, pancreatic, and pituitary tumors. The outcome for patients with the MEN-2a syndrome is also generally good, but the possibility exists for recurrence of medullary thyroid carcinoma and pheochromocytoma. For patients with the Carney complex, prognosis depends on the frequency of recurrences of cardiac and skin tumors.

Skin Cancer (Melanoma, Basal Cell Carcinoma, Squamous Cell Carcinoma)

Melanoma is thought to be the most common skin cancer in children, followed by basal cell and squamous cell carcinomas. The incidence of melanoma in children and adolescents represents approximately 1% of the new cases of melanoma that are diagnosed annually in the United States. In all instances, melanoma in the pediatric population is similar to that of adults in relation to site of presentation, symptoms, description, spread, and prognosis.

The most common cause of skin cancer of any type is exposure to the ultraviolet (UV) portion of sunlight. Other causes may be related to chemical carcinogenesis, radiation exposure, immunodeficiency, or immunosuppression. The person who is most likely to develop a melanoma is easily sunburned, has poor tanning ability, and generally has light hair, blue eyes, and pale skin. Worldwide, there is an increasing incidence of both melanoma and nonmelanoma skin cancers. Melanoma presents as a relatively flat, dark-colored lesion, that may enlarge, penetrate the skin, or metastasize.

Melanomas may be congenital (present at birth). They are sometimes associated with large congenital black spots known as melanocytic nevi, which may cover the trunk and thigh. Children with hereditary immunodeficiencies have an increased lifetime risk of developing melanoma.

Individuals with atypical moles, which include raised lesions (that may or may not bleed) and various color hues (brown, tan, pink, black) are at an increased risk of having melanoma and having children affected by these premalignant lesions. Basal cell carcinoma generally appears as a raised lump or ulcerated lesion, usually in areas with previous sun exposure. Squamous cell carcinomas are usually reddened lesions with varying degrees of scaling or crusting; they have an appearance similar to eczema, infections, trauma, or psoriasis.

Basal and squamous cell carcinomas are generally curable with surgery alone, but the treatment of melanoma requires greater consideration because of its potential for metastasis. Surgery for melanoma depends on the size, site, level of invasion, and metastatic extent or stage of the tumor. Prognosis for melanoma in children and adolescents is similar to that for adults with similar stage disease, with the prognosis depending on the tumor thickness and the extent of spread at the time of diagnosis. (Refer to Adult Skin Cancer Treatment for more information.)

Clear Cell Sarcoma of Tendon Sheaths

Malignant melanoma of soft parts was described once as "clear cell sarcoma of tendons and aponeuroses." More than 95% of these tumors present in the arms and legs. Survival with this tumor may depend on tumor size and other microscopic features. Cytogenetic studies of these tumors have noted a specific chromosomal abnormality. Treatment includes surgical removal, radiation therapy, and chemotherapy similar to that given for soft tissue sarcomas.

Cancer of Unknown Primary Site

Cancers of unknown primary sites present as metastatic cancers for which precise primary tumor sites cannot be determined. As an example, lymph nodes at the base of the skull may enlarge in relationship to a tumor that may be on the face or the scalp but not evident by physical examination or by x-ray. Thus, the extent of the disease may be evident, but a primary site is not evident.

For all patients who present with tumors of unknown primary sites, treatment should be considered in relation to the pathology of the tumor, and treatment appropriate for the general type of cancer should be initiated, regardless of the site or sites of involvement. Depending on histologic findings, symptoms, and the extent of tumor, appropriate and relevant chemotherapy and radiation therapy treatments should be initiated as early as feasible.






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