A Rare Cancer in Children

It is always heartbreaking when children have cancer. Usually cancer in children affects the blood cells or brain. A very rare cancer in children is rhabdomyosarcoma. Where is this cancer located in a child's body?

Fewer than 60 children are diagnosed with rhabdomyosarcoma in the UK each year. About the same number in the United States. Most of them are younger than 10 years old. It's more common in boys than girls.
Rhabdomyosarcoma is the most common of the soft tissue sarcomas in children. These tumors develop from muscle or fibrous tissue and can grow in any part of the body.
The most common areas of the body to be affected are around the head and neck, the bladder or the testes. Sometimes tumours are also found in a muscle or a limb, in the chest or in the abdominal wall. If the tumour is in the head or neck region, it can occasionally spread into the brain or the fluid around the spinal cord.

What causes this disease is unknown. Children who have rare genetic disorders are more prone to have rhabdommyosarcoma.

                               Image of rhabdomyosarcoma that has been removed from a child's body.

The images of the children with this cancer are very disturbing so they will not be displayed.

The signs and symptoms will depend on the part of the body that's affected by the rhabdomyosarcoma. The most common sign is a swelling or lump.

• If the tumor is in the head area, it can sometimes cause a blockage (obstruction) and a discharge from the nose or throat. Occasionally, an eye may appear swollen and protruding.
• If the tumor is in the abdomen (tummy), your child may have discomfort in the abdomen and difficulty going to the toilet.
• If the tumor is in the bladder, your child may have blood in the urine and difficulty passing urine.  

A variety of tests and investigations may be needed to diagnose a rhabdomyosarcoma. A small operation may be needed to remove a sample from the tumour to be looked at under a microscope. This called a biopsy. It's usually done under a general anaesthetic.
Various tests may be done to check the exact size of the tumour and whether it has spread to any other part of the body. These may include:

• a chest x-ray to check the lungs
• an ultrasound
• CT or MRI scans
• blood and bone marrow tests.

Any tests and investigations that your child needs will be explained to you. The booklet A parent's guide to children's cancer gives details of what the tests and scans involve.

Rhabdomyosarcomas are rare tumours and should be treated at specialist centres. 
Treatment depends upon the size of the tumour, its position within the body, and whether it has spread. Treatment of rhabdomyosarcoma usually includes surgery, radiotherapy or chemotherapy, or a combination of these treatments.

Surgery

If at all possible, surgery will be used to remove the tumour. Chemotherapy, using a combination of drugs, is often given before surgery to shrink the tumour. Radiotherapy may also be given to the area of the tumour, particularly if it cannot be completely removed by surgery.

Chemotherapy

If the tumour cannot be removed with surgery, treatment will usually involve a combination of chemotherapy and radiotherapy. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells and is usually given every three weeks. It may be given to shrink the tumour before surgery or with radiotherapy when the tumour can't be removed by surgery. The drugs used and the length of treatment depends on the type and stage of the rhabdomyosarcoma.

Radiotherapy

Radiotherapy treats cancer by using high-energy rays, which destroy the cancer cells while doing as little harm as possible to normal cells. It's given to the area where the rhabdomyosarcoma occurs.

Side effects of treatment 

Treatment for rhabdomyosarcoma often causes side effects, and your child’s doctor will discuss this with you before treatment starts. Any possible side effects will depend on the particular treatment being given and the part of the body that's being treated.
Chemotherapy can make your child feel better by relieving the symptoms of the cancer, but it can sometimes have side effects such as feeling sick (nausea) and being sick (vomiting), hair loss, an increased risk of infection, bruising and bleeding, tiredness and diarrhoea.

Late side effects

A small number of children may develop side effects many years after their treatment for a rhabdomyosarcoma. Long-term side effects depend on the type of treatment used, and may include a possible reduction in bone growth, infertility, a change in the way the heart and the kidneys work, and a slight increase in the risk of developing another cancer in later life.
Your child’s doctor or nurse will talk to you about any possible late side effects. There is more detailed information about these late side effects in the booklet A parent’s guide to children’s cancer.

                                        Mayo Clinic Oncologist Explains Rhabdomyosarcoma

                                       Holly's Journey Dealing With This Rare Cancer

http://www.macmillan.org.uk/Cancerinformation/Cancertypes/Childrenscancers/Typesofchildrenscancers/Rhabdomyosarcoma.aspx

Wilm's Tumor In Children

Wilm's tumor is a rare type of cancer that is found mainly in children. Another name for Wilm's tumor is nephroblastoma and it is the most common cancer of the kidneys.  The age range of a child is 3-4 years old is when the Wilms tumor usually develops.  This specific tumor is usually seen on just one of the kidneys, but can be seen in both.

                                                     Wilms Tumor in kidneys of a child

Approximately 500 cases are seen every year in the United States. Fortunately, this type of tumor is highly responsive to treatment.

Wilms' tumor is a malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated muscle, cartilage, bone, fat tissue, fibrous tissue. The tumor is compressing the normal kidney parenchyma.

The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy (rhabdomyosarcomatous Wilms).

Wilms' tumors may be separated into 2 prognostic groups based on pathologic characteristics:

• Favorable - Contains well developed components mentioned above
• Anaplastic - Contains diffuse anaplasia (poorly developed cells)

The symptoms that are typical of Wilm's tumor are an abnormally large abdomen, abdominal pain,fever,nausea and vomiting,blood in the urine and high blood pressure in some cases.

The diagnosis of this tumor requires several tests.

• A physical examination. The doctor will look for possible signs of Wilms' tumor.
• Blood and urine tests. Blood tests can't detect Wilms' tumor, but they can provide your child's doctor with an overall assessment of your child's health.
• Imaging tests. Imaging tests that create pictures of your child's kidneys can help your doctor determine whether your child has a kidney tumor. Imaging tests may include ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI).
• Surgery. If your child has a kidney tumor, your doctor may recommend removing the tumor or the entire kidney to determine if the tumor is cancerous. The removed tissue is analyzed in a laboratory to determine whether cancer is present and what types of cells are involved. This surgery may also serve as treatment for Wilms' tumor.

Once your child's doctor has diagnosed Wilms' tumor, he or she works to determine the extent (stage) of the cancer. Your child's doctor may recommend a chest X-ray, chest CT scan, chest MRI and bone scan to determine whether the cancer has spread beyond the kidneys. 

The growth of the cancer is usually described in Stages.  The following are the stages for Wilm's tumor.

• Stage I. The cancer is found only in one kidney, and generally can be completely removed with surgery.
• Stage II. The cancer has spread to the tissues and structures near the affected kidney, such as fat or blood vessels, but it can still be completely removed by surgery.
• Stage III. The cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen, and it may not be completely removed by surgery.
• Stage IV. The cancer has spread to distant structures, such as the lungs, liver, bones or brain.
• Stage V. Cancer cells are found in both kidneys.

·         Standard treatment for Wilms' tumor is surgery and chemotherapy. The stage of the tumor and appearance of the cancer cells under a microscope help determine whether your child also needs radiation therapy. At this point, your doctor may tell you the tumor appears to be either favorable or unfavorable (anaplastic) — the histology of the tissue. Children whose tumors have a favorable histology have better survival rates. However, many children with unfavorable histology also have good outcomes.

·         Because this type of cancer is rare, your doctor may recommend that you seek treatment at a children's cancer center that has experience treating this type of cancer.

Professor Frank Friedman explains Wilm's Tumor Kidney Cancer in Children

More information : www.mayoclinic.com, 

 

Chordoma Tumor Found In Children And Young Teens

Chorodoma tumors can affect any age but in children and adolescents tumors behave very aggressively and have high levels of mitotic activity, hypercellularity, and pleomorphism;13 some authors have suggested that the prognosis for patients younger than 40 years of age is significantly better than that for the older population  survival rates for the younger is 10 years.
Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the base of the skull or at end of the spine (in the sacrum or the coccyx [the tail bone]) with about equal frequency. The cells that give rise to chordoma come from the notochord. The notochord is an important structure in the early embryo that disappears before birth. However, even after birth, some cells from the notochord remain in bones at the base of the skull, in vertebrae, and in the tail bone. Rarely, one of these cells, which are called notochord remnants, undergoes changes that give rise to a chordoma.

                                          

In the United States, there are around 300 new cases of chordoma diagnosed each year. Based on this statistic, the annual incidence of chordoma is approximately one new case per million people per year. The incidence in Europe appears to be similar, but is unknown in other continents. Chordomas account for about 3% of all bone tumors and about 20% of primary spinal tumors. Chordomas are the most common tumor of the sacrum and cervical spine.

What are the signs and Symptoms?

The most common signs of chordoma are pain and neurological changes. Skull base chordomas most often cause headache, neck pain, diplopia (double vision), or facial nerve palsy (paralysis of facial muscles). Chordomas of the spine and sacrum can cause changes in bowel and/or bladder function, pain, aching, tingling, numbness, or weakness of the arms and legs. Often sacral chordomas do not cause symptoms until the tumor is quite large and sometimes a lump is the first sign of a sacral chordoma.

What is the treatment for Chordomas?

Surgery
Currently, surgery is the first-line treatment for chordomas. Complete resection (removing the entire tumor) during the first surgery provides the best chances for local control and long-term survival.To achieve a complete resection, aggressive surgery is often required and can lead to significant complications or side-effects.
The goal of surgery should be to remove as much of the tumor as possible without causing unacceptable harm. Because outcome and prognosis are largely dependent on the success of the initial surgery, before having any operation it is very important to get multiple opinions from surgical teams who have experience treating chordomas on a regular basis. Some medical centers have multidisciplinary teams of experts who review cases and can help create a coordinated treatment plan for patients with chordomas.
Treatment of patients with chordoma of the skull base is a challenge for neurosurgeons. Because of the origin of the tumor from the bone at the base of the skull, exceptionally complete resection can be achieved. Microscopic total removal of chordoma frequently is followed by the finding of residual tumor in the postoperative computerized tomography and MR images. The recurrence rate, even after radical resection, remains high. The deep localization of chordomas at the middle of the skull base makes surgical access to these tumors difficult; nevertheless, many approaches lead to the clival from the superior, anterior or lateral view. The patterns of spread of the skull base chordoma preclude the use of a single surgical approach. Approaches to chordomas of the skull base should be based on the characteristics of growth in each case, and sometimes two or more skull base procedures may be necessary to achieve a radical removal. Extensive excision has an important role in the treatment of skull base chordoma; however, sometimes unacceptable procedure-related morbidity may occur. Currently, many authors consider that most cases of chordoma should be treated with resection. The average survival for patients with untreated chordoma is estimated to be 28 months after the onset of symptoms.32 Survival after surgery or radiation therapy, or both, ranges from 3.6 to 6.6 years, and all tumors are seen to recur with time.^[ On average, recurrence is observed from 2 to 3 years after primary treatment, but sometimes the tumor recurs more than 10 years after initial treatment.


Radiotherapy
In many cases, radiation therapy following surgery is recommended and can improve chances of local control and survival. Because chordomas do not grow rapidly, high doses of radiation are required to kill the tumor cells. Chordomas that are close to critical structures (arteries, brain, brainstem, cranial nerves, dura, spinal nerves), often limit the dose of radiation that can be safely delivered to the tumor. In most cases, proton beam radiation can maximize the dose of radiation to the tumor, while sparing adjacent critical structures. Intensity modulated radiation therapy (IMRT), stereotactic radiosurgery (CyberKnife® or Gamma Knife®) and carbon-ion radiotherapy are also sometimes used to treat chordoma.

You may be wondering about chemotherapy to kill the tumor cells,but it has been found that this treatment has not been very effective in most patients with chordoma tumors.  There is hope because clinical trials are going on to find a successful chemotherapy drug.

                                        Dr. Chandranath Sen lecturing about Sugical treatment of 
                                              Chordoma tumors
 
For more information : /www.chordomafoundation.org/chordoma/

Astrocytoma Brain Tumors

What is an Astrocytoma brain  tumor?  Astrocytoma tumors are a form of glioma with star-shaped cells. Glioma is is a type of tumor that starts in the brain or spine. It is called a glioma because it arises from glial cells. The most common site of gliomas is the brain.

                                                                                                                                       
Astrocytoma tumors often grow very slowly or not at all for long periods of time. Therefore, close observation rather than treatment is possible in some cases (especially ones associated with neurofibromatosis ). They may occur in many parts of the brain, but most commonly in the cerebrum. They occur less commonly in the spinal cord. People of all ages can develop astrocytomas, but they are more prevalent in adults, particularly middle-aged men. Astrocytomas in the base of the brain are more prevalent in children or younger people and account for the majority of children’s brain tumors. In children, most of these tumors are considered low-grade, while in adults most are high-grade.

General symptoms of an astrocytoma tumor are a result of growing pressure inside the skull. These symptoms include headache, vomiting and mental status changes. Other symptoms, such as drowsiness, lethargy, obtuseness, personality changes, disordered conduct and impaired mental faculties show up early in about one out of every four patients with malignant brain tumors.

In young children, the growing pressure of an astrocytoma tumor inside the skull may enlarge the head. Changes (such as swelling) may be observed in the back of the eye, where the blind spot is. Usually there are no changes in temperature, blood pressure, pulse or respiratory rates except just before death. Seizures are more common with meningiomas, slow-growing astrocytomas and oligodendrogliomas than with malignant gliomas.

Symptoms of an astrocytoma tumor vary depending on what part of the brain (or which glands or nerves) are affected by the tumor. Sometimes the nature of the seizures can help determine the location of the brain tumor.

Astrocytomas are generally classified (graded) into one of three types: Low grade astrocytomas, anaplastic astrocytomas and glioblastomas. Low grade astrocytomas account for 10 percent of astrocytomas. These tumors are typically slow growing and may not require specific treatment at the time of diagnosis. Many patients with low grade astrocytomas live for prolonged periods of time after their diagnosis. However, these tumors often advance into the higher grades and more rapidly growing forms of brain gliomas. Anaplastic astrocytomas and glioblastomas are the most aggressive and, unfortunately, the most common astrocytomas. Glioblastomas are fast growing astrocytomas that contain areas of dead tumor cells. In adults, glioblastoma occurs most often in the cerebrum, especially in the frontal and temporal lobes of the brain.

Diagnosis

A neurologic evaluation should be conducted if a patient has slowly increasing signs of mental dysfunction, new seizures, persistent headaches or evidence of pressure inside the skull, such as vomiting or swelling or protrusion of the blind spot at the back of the eye.

A neurologist (a doctor who has received special additional training in the diagnosis and treatment of disorders of the brain, spinal cord and nerves)performs a complete examination, which may include a magnetic resonance imaging (MRI) scan, a computed.

Treatment Options
Treatment options include surgery, radiation, radiosurgery, and chemotherapy. The main goal of surgery is to remove as much of the tumor as possible without injuring brain tissue needed for neurological function (such as the ability to speak, walk, motor skills, etc.). However, high-grade tumors often have tentacle-like structures that invade surrounding tissues, making it more difficult to remove the entire tumor. If the tumor cannot be completely removed, surgery can still reduce or control tumor size. In most cases, surgeons open the skull through a craniotomy to best access the tumor site. The goal of radiation therapy is to selectively kill tumor cells while leaving normal brain tissue unharmed. In standard external beam radiation therapy, multiple treatments of standard-dose "fractions" of radiation are applied to the brain. Each treatment induces damage to both healthy and normal tissue. By the time the next treatment is given, most of the normal cells have repaired the damage, but the tumor tissue has not. This process is repeated for a total of 10 to 30 treatments, depending on the type of tumor. This additional treatment provides some patients with improved outcomes and longer survival rates.
Radiosurgery is a treatment method that uses computerized calculations to focus radiation at the site of the tumor while minimizing the radiation dose to the surrounding brain. Radiosurgery may be an adjunct to other treatments, or it may represent the primary treatment technique for some tumors
Patients undergoing chemotherapy are administered special drugs designed to kill tumor cells. Although chemotherapy may improve overall survival in patients with the most malignant primary brain tumors, it does so in only about 20 percent of patients. Chemotherapy is often used in young children instead of radiation, as radiation may have negative effects on the developing brain. The decision to prescribe this treatment is based on a patient’s overall health, type of tumor, and extent of the cancer. Before considering chemotherapy, you should discuss it with your doctor, as there are many side effects.
Because traditional treatment modalities are unlikely to result in a prolonged remission of malignant astrocytomas, researchers are presently investigating a number of promising new treatments including gene therapy, highly focused radiation therapy, immunotherapy and novel chemotherapies. A number of new treatments are being made available on an investigational basis at centers specializing in brain tumor therapies.

                                           Astrocytoma Explained by Dr. Mark Atlas

For more information: http://www.cedars-sinai.edu/Patients/Health-Conditions/Astrocytoma-Brain-Tumors.aspx

http://www.aans.org/Patient%20Information/Conditions%20and%20Treatments/Astrocytoma%20Tumors.aspx