rare & unusual cancers of childhood
general information the tumours discussed in this summary are many and different, and the discussion is arranged in descending order from tumours of the head and neck to tumours of the urinary system and skin. all of these cancers are rare enough that most paediatric hospitals might see fewer than two cases in a year. most of these tumours are more frequent in adults with cancer; thus, much of the information about these tumours may also be sought through sources relevant to adults with these tumours. head and neck cancers head and neck cancers include cancers of the nose and throat, thyroid tumours, mouth cancer, salivary gland cancer, cancer of the larynx (or voice box), and upper respiratory tract. 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 tumour 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 tumour. the tumour 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. studies show that combining chemotherapy with radiation therapy is the most effective treatment for this tumour. refer to the summary on nasopharyngeal cancer treatment for more information. esthesioneuroblastoma esthesioneuroblastoma (olfactory neuroblastoma) is a very rare, small tumour that begins in the olfactory bulb (the organ responsible for the sense of smell) located in the front part of the brain. most children have a tumour in the nose or throat at the time of diagnosis. the tumour may extend into the eyes, sinuses, and the front part of the brain. esthesioneuroblastoma occurs more often in boys and usually appears during adolescence. the disease seldom spreads to other parts of the body. treatment for this cancer is usually surgery and radiation therapy; chemotherapy may also be used. thyroid tumours tumours 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 tumours 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. thyroid carcinomas are rare and occur most often in girls. this cancer usually appears as a lump or mass in the thyroid with possible swelling of the lymph glands in the neck. surgery is the treatment required for all thyroid tumours. this is usually removal of all or nearly all of the thyroid and nearby lymph nodes in the neck. treatment with a radioactive form of iodine is given after surgery to destroy cancer cells and thyroid tissue that remain. after surgery and treatment with radioactive iodine, hormone replacement therapy must be given to compensate for the lost thyroid hormone. regular checkups 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. thyroid tumours that recur (come back) are usually treated with radioactive iodine. even patients with tumour that has spread to the lungs may expect no decrease in life span after appropriate treatment. (refer to the summary on adult thyroid cancer treatment for more information.) oral (mouth) cancers oral cancer in children or in adolescents is extremely rare. most oral tumours are benign (not cancer). malignant tumours include lymphomas (often burkitt's lymphoma) and sarcomas (soft tissue tumours). oral squamous cell carcinoma (cancer of the thin, flat cells lining the mouth) is the most common type of oral cancer in adults, but is rare in children; adolescents (teens) with oral squamous cell carcinoma should be screened for a condition called fanconi's anemia. treatment of oral cancer in children may include surgery, chemotherapy, and radiation therapy. (refer to the summaries on adult oropharyngeal cancer treatment and lip and oral cavity cancer treatment for more information). salivary gland tumours salivary glands are the parts of the mouth and throat that produce saliva. many of the tumours in these areas arise in the parotid gland. about 15% of these tumours may arise in the submandibular glands or in the minor salivary glands under the tongue and jaw. these tumours 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 tumours may occur after radiation therapy for treatment of primary leukaemia or solid tumours. complete surgical removal is the treatment of choice whenever possible, with additional use of radiation therapy and chemotherapy. prognosis (outcome) for patients with these tumours is generally good. (refer to the summary on adult salivary gland cancer treatment for more information.) laryngeal cancer and papillomatosis benign and especially malignant (cancerous) tumours of the larynx (voice box) are rare. malignant tumours may be associated with benign tumours such as polyps and papillomas. these tumours may cause hoarseness, difficulty swallowing, and enlargement of the lymph nodes of the neck. rhabdomyosarcoma (a malignant tumour of muscle tissue) is the most common malignant tumour of the larynx in the paediatric 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, but may recur after treatment. these tumours 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. treatment includes laser surgery. (refer to the summary on adult laryngeal cancer treatment for more information.) respiratory tract cancer with chromosome 15 changes the respiratory tract includes the nose, throat, larynx, trachea, and lungs. respiratory tract cancer may be caused by a specific chromosome change. every cell in the body contains dna (genetic material stored inside chromosomes) that determines how the cell looks and acts. cancer may develop when part of the dna from chromosome 15 moves to another chromosome, or when chromosome 15 is broken. this type of cancer may appear in parts of the airway or in other places along the midline of the body, including the thymus, the area between the lungs, and the bladder. it usually cannot be cured. thoracic cancers thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid tumours, pleuropulmonary blastoma, oesophageal tumours, thymomas, tumours of the heart, and mesothelioma. these thoracic cancers are discussed below. breast cancer most tumours that involve the breast during childhood are benign (noncancerous) fibroadenomas that can be watched for change without the need for biopsy. rarely, such breast tumours will show malignant change with sudden, rapid growth. these are called phyllodes tumours, and require biopsy or surgical removal without mastectomy. other types of breast cancer have been reported in both males and females younger than 21 years. there is an increased lifetime risk of breast cancer in patients who were treated for any cancer with radiation therapy to the chest area, including female survivors of hodgkins lymphoma. mammograms should start at age 25 or 10 years after radiation therapy to the chest, whichever came last. treatment options include radiation, chemotherapy, and surgery for children and adolescents with breast cancer. breast tumours may also occur as cancers that have spread from other types of cancer such as leukaemia, rhabdomyosarcoma, other sarcomas, or lymphoma, particularly in patients infected with the human immunodeficiency virus (hiv).(refer to the summary on adult breast cancer treatment for more information.) bronchial tumours bronchial tumours (which are slow-growing cancers in the trachea or large bronchi, the large airways of the lung), are most often carcinoid tumours in children. symptoms include cough and spitting up blood, and can sometimes be confused with asthma symptoms like wheezing, resulting in delayed diagnosis. primary treatment is surgery to remove the tumour and lymph nodes and vessels where cancer may spread. the prognosis for most bronchial tumours in children is excellent, even when the cancer has spread to nearby areas. rarely, carcinoid tumours develop as aggressive(fast-growing) cancers that are more likely to spread to other areas of the body by the time they are diagnosed. for bronchial carcinoid tumours, 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 tumours that usually occur under the tissue covering the lungs. the tumours may recur or spread, in spite of surgical removal. responses to chemotherapy have been reported. radiation may be used when the tumour cannot be surgically removed. a family history of cancer in close relatives has been noted for many young patients affected by this tumour. oesophageal tumours cancer of the oesophagus (the muscular tube through which food passes from the throat to the stomach) is rare in the paediatric age group. symptoms are related to difficulty in swallowing, and associated weight loss. treatment options for oesophageal cancer include either radiation therapy or chemotherapy. prognosis generally is poor for this cancer, which rarely can be completely removed by surgery. (refer to the summary on adult oesophageal cancer treatment for more information.) thymomas and thymic carcinoma a cancer of the thymus (an organ in the chest, behind the breastbone) is not considered a thymoma (cancer) or a thymic carcinoma unless there are cancerous changes of the epithelial cells that cover the organ. the term thymoma usually describes cancers that do not have obvious changes in the epithelial cells. thymic carcinoma has clear-cut changes of the epithelial cells. other tumours that involve the thymus gland include lymphoma (cancer that arises in cells of the lymphatic system) and germ cell tumours (tumours that begin in cells that give rise to sperm or eggs); these tumours are not true thymomas or thymic carcinomas. thymomas and thymic carcinomas 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, addisons disease, and panhypopituitarism can also be associated with a diagnosis of thymoma or thymic carcinoma. cancer of the thymus may be caused by a specific chromosome change. every cell in the body contains dna (genetic material stored inside chromosomes) that determines how the cell looks and acts. cancer may develop when part of the dna from chromosome 15 moves to another chromosome, or when chromosome 15 is broken. this type of cancer may appear in the thymus or in other places along the midline of the body, including parts of the airway, the area between the lungs, and the bladder. it usually cannot be cured. thymomas and thymic carcinomas 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 tumours 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 or thymic carcinoma, 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 or thymic carcinoma usually is poor, although significantly higher survival rates have been reported for patients with tumours that have not spread to the surrounding areas. (refer to the summary on adult thymoma and thymic carcinoma treatment for more information.) tumours of the heart primary tumours of the heart may include benign (noncancerous) and malignant (cancerous) teratoma (a tumour made up of a mixture of tissues), rhabdomyosarcoma (a tumour of muscle tissue), hemangioma (a usually benign tumour 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. benign tumours of heart muscle (rhabdomyomas) usually shrink and go away on their own. mesothelioma mesothelioma, an extremely rare cancer in children, can involve the tissue coverings of the lung, the heart, or the abdominal organs. these tumours 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 tumours 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 the summary on 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 tumours 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 patients gender. paediatric 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 tumours can involve the kidneys, lungs, and bones. surgical removal should be attempted but may not always be possible if the tumour has spread widely. additional treatment may include the use of an artificial hormone that blocks the masculinizing effects of the tumour. the prognosis is generally excellent for patients who have small tumours that have been completely removed by surgery, but prognosis can be poor for patients who have large primary tumours or metastatic disease (disease that has spread to other parts of the body) at diagnosis. (refer to the summary on 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 tumour 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. 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 tumour. (refer to the summary on 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 paediatric age group, little information exists regarding treatment outcomes of children. (refer to the summary on adult gastric cancer treatment for more information.) cancer of the pancreas tumours of the pancreas (a gland in the abdomen that makes pancreatic juices and produces hormones) are rare in children and adolescents. tumours included within the general category can arise at any site in the pancreas. most pancreatic tumours do not secrete hormones, although some tumours secrete insulin, which can lead to symptoms of weakness, fatigue, hypoglycemia, and coma. if a tumour 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 paediatric patients, the effectiveness of radiation therapy is not known. chemotherapy may be useful for treatment of localised or metastatic pancreatic carcinoma, although few cases have been successfully treated. response rates and survival rates generally are not good. (refer to the summary on adult pancreatic cancer treatment for more information.) colorectal cancer cancer of the large bowel is rare in the paediatric age group: one person per one million younger than 20 years in the united states annually. in children, more than half of colon tumours begin on the right side, compared with adults, who have more colon tumours on the left side. colon cancer in children is often linked to a family colorectal cancer syndrome, or inherited pattern. 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 tumours). 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 tumour. changes in bowel habits are associated with tumours of the rectum or lower colon. tumours 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 tumour that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumour cells within the abdominal cavity. colorectal carcinoma is rarely diagnosed in a paediatric 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 tumour 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 tumour 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 tumours, in conjunction with chemotherapy. (refer to the summaries on adult colon and rectal cancer treatment for more information.) carcinoid tumours carcinoid tumours 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 tumours of the large bowel or stomach becomes more complicated and requires treatment similar to that given for colorectal cancer. (refer to the summary on gastrointestinal carcinoid tumours treatment for more information.) genital/urinary tumours genital/urinary tumours 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 tumour are often prone to the development of other cancers. bladder cancer in adolescents may develop as a late effect of certain chemotherapy drugs given for other childhood tumours or leukaemia. (refer to the summary on adult bladder cancer treatment for more information.) ovarian cancer most ovarian tumours in children are benign (noncancerous). the most common cancers that affect the ovaries are of germ cell origin (beginning in cells that give rise to sperm or eggs); these are more common in children than in adults. common symptoms of ovarian cancer include painful periods and pain in the abdomen. treatment is stage related and may include surgery, radiation therapy, and chemotherapy. (refer to the summaries on childhood extracranial germ cell tumours treatment, ovarian epithelial cancer treatment, ovarian germ cell tumours treatment, or ovarian low malignant potential tumours treatment for more information.) carcinoma of the cervix and vagina cancer of the cervix and vagina is very rare in children and adolescents. most young patients diagnosed with cancer of the cervix or vagina were exposed to a drug called diethylstilbestrol (des) before birth when it was given to their mothers. des was given to pregnant women between 1945 and 1970 to keep them from losing their babies (miscarriage). the most common symptom of cancer of the cervix or vagina is vaginal bleeding. treatment includes surgery followed by radiation and possibly chemotherapy. 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 one endocrine organ at the same time (endocrine tissue secretes hormones). these changes may include hyperplasia (overgrowth of tissue) or benign (noncancerous) tumours. the distinct involvement of multiple glandular structures are referred to as men-1 (werners syndrome) which may involve tumours 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 phaeochromocytoma; men-2b is associated with medullary thyroid carcinoma, parathyroid hyperplasia, and adenomas as well as phaeochromocytoma, mucosal neuromas, and ganglioneuromas. an additional complex is referred to as the carney complex, which is associated with heart and skin tumours. patients with the men-2b syndrome may have a slender body build, long and thin extremities, a high arch palate, and funnel chest (sunken chest) or an abnormally high arch in the foot. the lips may appear thickened because of tumours in the mucous membranes. 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 tumours. the outcome for patients with the men-2a syndrome is also generally good, but the possibility exists for recurrence of medullary thyroid carcinoma and phaeochromocytoma. medullary thyroid cancer in children with men-2b may be difficult to cure. for patients with the carney complex, prognosis depends on how often heart and skin tumours recur. 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 paediatric 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 tumour. chordoma chordoma is a very rare type of bone tumour that may develop along the spine at any point from the base of the skull to the tailbone. chordomas start in clusters of cells leftover from spinal column development in the embryo. these cells normally disappear, but very rarely they remain and grow into tumours. in children and adolescents, especially girls, chordomas often develop in the clivus, a bone at the base of the skull. symptoms vary and may include pain and nerve trouble. when chordoma recurs, it usually comes back in the same area, but may appear in the lungs or other areas of bone. standard treatment includes surgery and radiation therapy. the best results are seen with proton beam therapy, a special kind of high-energy radiation that is different from an x-ray. cancer of unknown primary site cancer can form in any tissue of the body and can spread from the primary site (the place where the cancer first began to grow) to other parts of the body. cancer that has spread from the place in which it started to other parts of the body is called metastatic cancer. metastatic cancer cells usually look like cells in the type of tissue where the cancer began. for example, breast cancer cells that spread to the lung look like breast cancer cells, not lung cancer cells. sometimes metastatic cancer cells are found in the body, but tests do not find a primary tumour. if cancer cells are found in the body but the place where the cancer started cannot be identified, the disease is called cancer of unknown primary site. treatment is based on what the cancer cells look like under a microscope, the patient's symptoms, and the extent of the cancer in the body. treatment is usually chemotherapy or radiation therapy.Relaterede Sundhed Artikler
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