indiacancer.org - Ewing's Tumors

Ewing's Tumors

		What are Ewing's tumors?
		How do Ewing's tumors present?
		How are Ewing's tumors detected?
		What are staging and grading?
		How are the Ewing's family of tumors treated?
		What are the side effects of treatment for the Ewing's family of tumors?
		What is the importance of follow up?

What are Ewing's tumors?

This is a family of tumors which includes the following:

Extra osseous Ewing's; a tumor growing outside the bone.

Ewing's tumor of bone.

PNET or primitive neuroectodermal tumor also known as peripheral neuroepithelioma.

Askin Rosai tumor or PNET of the chest wall. These are extremely rare tumors in which cancer cells are found in the bones and the soft tissues. These are diseases which are commonly seen in teenagers.

How do Ewing's tumors present?

The usual presentation is in the form of:

Tenderness in the bone, stiffness or pain.

How are Ewing's tumors detected?

When these symptoms are persistent for no obvious reason and there is no history of trauma, then the doctor may order x-rays and other tests like CT scan.

The doctor also may perform a biopsy from the affected area. This biopsy is the removal of a piece of bone or soft tissue. Usually this is known as a 'J' needle or a Jamshidi needle biopsy. This tissue will be examined under a microscope to validate the presence or absence of cancer cells.

What are staging and grading?

	These are terms with which you must become familiar, if you are dealing with any form of cancer. When we stage a cancer, we try to establish carefully degree of spread of the cancer and if indeed there is spread, to what extent and involving which organs. The grading of a cancer is a microscopic issue, in which the pathologist lets you know how aggressive this particular type of cancer is ; well differentiated means less aggressive, moderately differentiated intermediately aggressive and poorly differentiated more aggressive. Both grading and staging help your clinician to establish the prognosis or how serious your disease is and what is going to be the likely outcome. Staging may require additional imaging tests such as CT Scan, MRI, Sonography of the abdomen, IVU, Bone scan or chest X-ray. At present, unfortunately, there is no standardized staging pattern for the Ewing's family of tumors. What is used instead is the fact that patients are grouped depending on whether the cancer is located in only one part of the body, which is known as a localized cancer or whether it has spread to many parts of the body, which is known as metastatic disease. Extra osseous Ewing's tumor is grouped using the rhabdomyosarcoma staging system because they are both soft tissue tumors. Rhabdomyosarcoma is a type of cancer affecting skeletal muscles or muscles which are voluntary and connected to the skeleton. The following groups are applicable for the Ewing's family of tumors:
		Localized. The cancer has not been shown to have spread beyond bone or are found only in the bone and nearby surrounding tissues.
		Metastatic. The cancer has spread to distant parts of the body, usually the lungs, other bones and bone marrow, which is a spongy tissue inside large bones responsible for production of red blood cells. The cancer may have also spread to the lymph nodes (Lymph nodes are small bean-shaped structures, which act as stations for drainage of lymph, a clear watery fluid, which courses through out the body in transparent, thin tubes called lymph channels or lymphatics,from various organs), or the central nervous system which comprises of the brain and spinal cord. However, the last one is quite uncommon. Recurrent.
	Recurrent Ewing's sarcoma or Ewing's variety of tumors are those which have come back after the primary treatment is over. It may have come back to where it originated initially or it may come back in other parts of the body.

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How are the Ewing's family of tumors treated?

	A lot depends on the patient's constitution, the grade of his disease and its staging. Patients are usually treated by a team of specialists which utilizes a multipronged approach. This includes an an orthopaedic surgeon, an oncosurgeon and a radiotherapist. The plan of treatment is tailored to fit a patient's requirements. If necessary, chemotherapy may become an add on. Treatment strategies. The bastions of treatment for Ewing's family of tumors are surgery, radiation or chemotherapy. Surgery is the commonest method employed and is used in certain cases in order to remove the tumor and some of the healthy tissues surrounding it in order to get a clear, safe margin. Surgery may also be used to remove residual disease after the effect of chemotherapy and radiotherapy. The alternative local therapy to surgery is radiation therapy. This involves the use of high energy, penetrative rays to destroy cancer cells. It affects cancer cells only in the zone treated. Radiation therapy is also employed for palliation i.e. control of symptoms alone in an advanced cancer. Teletherapy or external radiation is given via a machine remote from the body while brachytherapy or internal radiation is given by implanting a radioactive source within the involved tissues. Patients may or may not require both modalities of radiation. Radiotherapy, after initial planning, usually takes just 5 to 10 minutes per day, 5 days a week for about 6 weeks. This time period is flexible depending upon the modality of radiation used. Besides local therapy, the best attempt to control cancer cells circulating in the body and lodged at places other than the bone and the soft tissues of the primary focus, is by using systemic therapy (therapy which affects the entire systems of the body) which is in the form of injections or oral drugs. This form of treatment is called chemotherapy. This form of treatment is given in a cyclical manner (each set of drugs is repeated usually after every 3-4 weeks). Chemotherapy can also be used in combination with surgery, radiotherapy or both, either before or after. In treating Ewing's family of tumors, surgery or radiation is most often used to remove the local tumor and chemotherapy is used for the systemic purpose. An add-on to all these therapies is myeloablative therapy with stem cell support. This is a very intense regimen of chemotherapy which destroys all cells that divide rapidly, which would include all blood cells, hair cells as well as malignant cells. Stem cells are self-renewing cells that create all other various types of blood cells. Stem cell support or peripheral blood stem cell rescue involves enriching the stem cells to increase the number of these important cells circulating in the blood, once chemotherapy has been given to kill the remaining tumor cells. Localized tumors of the Ewing's family. The following options may be entertained:
		Chemotherapy followed by radiation.
		Combination chemotherapy followed by surgery with or without radiation.
		Post surgical chemotherapy with or without stem cell transplant.
		Intensified chemotherapy.
	Metastatic tumors of the Ewing's family. The treatment options which may be exercised are as follows:
		Combination chemotherapy followed by radiation and/or surgery.
		High dose chemotherapy with or without radiation plus additional stem cell support.
		Intensive chemotherapy with multiple chemotherapy drug combinations.
	Recurrent tumors of the Ewing's family. Treatment options may be one of the following:
		Depending on where the cancer has recurred and how it was treated before, the treatment can be planned out.
		All those patients who have not received prior chemotherapy may get it now. The same applies for radiation.
		Radiation may also be given for palliation and to reduce the symptomatology of advanced disease. Surgery may be used to excise tumor that has spread to organs such as the lungs.

What are the side effects of treatment for the Ewing's family of tumors?

	Unfortunately treatment for cancer does cause damage to the healthy tissues. Please ask the clinician about any possible side effects before any form of treatment commences. The effects of radiation depend upon the dose of radiation, the size of the area radiated, and the number and size of each fraction. The commonest side effect is extreme fatigue. Although bed rest is good, most radiotherapists advise the patient to be as mobile as possible. Another common problem is that of radiation dermatitis in which the skin covering the radiated area becomes red, dry, itchy, and may show signs of scaling off. This will slowly settle down after radiation ceases, but there may be a permanent 'bronzing' of the skin. It is important to note here that this skin problem is associated only with teletherapy or external radiation therapy. Radiation also may cause nausea and vomiting, diarrhea, urinary discomfort. There may also be a fall in the white blood cells, which are needed by humans to fight infection. Usually the radiotherapist can suggest the drugs and diet necessary to alleviate such problems. The various drugs in chemotherapy cause a varied spectrum of side effects. The severity and variability of these symptoms are very individualistic, changing from patient to patient. Some of the common side effects are:
		Skin rashes.
		Loss of hair.
		Diarrhea.
		Vomiting.
		Tingling and numbness in the fingers and toes.
		Hearing loss.
	Most are temporary and recede after therapy is over. Hair growth gradually starts after cessation of chemotherapy. The medical oncologist will usually be able to reduce the severity and spectrum of these side effects. The psychology of the patient is very important during therapy. Better psychologically prepared the patient is, better are his chances of having decreased side effects. The patient should drink large quantities of water while the therapy is in progress, and preferably avoid uncooked or raw food. Water should be filtered or boiled. Any relative or person who has any sort of infection such as common cold should be asked to desist from coming close to the patient.

What is the importance of follow up?

The importance of followup cannot be overemphasized. It is a fatal fallacy to assume on the patient's part that once the primary or the first phase of treatment is over, then he or she is cured. It must be stressed here that cancer has a notorious proclivity of coming back again and again. The only way to fight this is to pick up any problem when it is still early, so that it can be nipped in the bud. And the only way to do this is to follow up when the doctor calls you for the next visit. Each such visit will include a detailed history, a physical examination (including an internal i.e. a rectal or vaginal examination), a chest x-ray, an ultrasonography or a CT scan, and various other blood studies as indicated.