indiacancer.org - The Soft Tissue Sarcomas

The Soft Tissue Sarcomas

		What are the Soft Tissues of the body?
		What is Adult Soft Tissue Sarcoma?
		How does Adult Soft Tissue Sarcoma present?
		How are Adult Soft Tissue Sarcomas detected?
		What is staging and grading?
		How are Soft Tissue Sarcomas treated?
		What are the side effects of treatments for Soft Tissue Sarcomas?
		What is the importance of follow up?

What are the Soft Tissues of the body?

The soft tissues of the body comprise the connective tissues which include the fasciae and the tendons, the vessels that carry the blood or lymph, the joints, the fat pads present beneath the skin and in between the organs and the muscles.

The main difference between sarcomas and carcinomas is that as a general rule sarcomas metastasize (spread) using the blood stream and this kind of spread is known as hematogenous spread, while carcinomas usually metastasize using the lymphatic system and the lymph nodes. Secondly, sarcomas although can be seen in various age groups but they have a predilection for appearing in the younger age groups and are generally more aggressive and symptomatic than carcinomas.

	What is Adult Soft Tissue Sarcoma?

This is a disease in which the malignant cells are produced from the soft tissue compartments of the body.

	How does Adult Soft Tissue Sarcoma present?

The usual presentation is in the form of a lump or a swelling in any part of the body, whether symptomatic or asymptomatic. By symptomatic or asymptomatic, it is meant that the lump may or may not be painful or may or may not give any pressure effect.

If the symptoms have suddenly arisen, or if the lump has suddenly appeared, or an old lump is suddenly showing signs of growing, then the patient has to undergo certain investigations suggested by the doctor.

	How are adult soft tissue sarcomas detected?

	The most important specific investigation after history and physical examination is over, is the performing of a biopsy which is the removal of a small piece of tissue from the swollen area to be subjected for scrutiny under the microscope of a pathologist. Sometimes if the culprit lump is not very large then during biopsy itself a complete excision (removal) of the lump may be performed in which case it is known as an excision biopsy. The other common test which can be done in place of an open biopsy is an FNAC or a fine-needle aspiration cytology in which just a small thin gauge needle is introduced into the lesion and a few cell samples are taken on to a slide which are cytofixed and sent for scrutiny. The other specific investigations in case of some fascial sarcomas would include:
		An x-ray or possibly two x-rays in two different planes of the offending part in order to find out the extent of the problem.
		A CT scan of the part and a CT scan of the areas to which the lesion is likely to metastasize such as the abdomen or the chest.
		An x-ray of the chest or a sonography of the abdomen.
	In case the lesion is located in the retroperitoneum, which is behind the organ sac of the abdomen then the clinician may also order an investigation called the IVU (intravenous urography) in order to look for compression of the ureters which are the tubes sending down urine from the kidneys to the urinary bladder.

	What is 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.

Stage 1A.The cancer is well differentiated or moderately well differentiated, and it is either near the surface or is deep and is less than 5 cm in size and has not spread to the lymph nodes or other parts of the body (lymph nodes are small bean-shaped organs which are present at the junctions along the lymphatic system which consist of thin transparent tubings transmitting a clear fluid called lymph, which is basically transporting white blood cells called lymphocytes, infection fighting cells in the body).

Stage 1B. The cancer is well differentiated or moderately well differentiated, is located near the surface, is greater than 5 cm in size, and has no signs of spread to either the nodes or to other parts of the body.

Stage 2A. The cancer is well or moderately differentiated and is deeply located and is greater than 5 cm in size and has not spread to the lymph nodes or other parts of the body.

Stage 2B. The cancer is poorly differentiated and/or undifferentiated either near the surface or deep, is less than 5 cm in size and has not spread to lymph nodes or other parts of the body.

Stage 2C. The cancer is poorly differentiated or undifferentiated, is near the surface and is greater than 5 cm in size but has not spread to lymph nodes or other parts of the body.

Stage 3. The cancer is poorly differentiated or undifferentiated, lies deeply situated and is greater than 5 cm in size but has not spread to lymph nodes or other parts of the body.

Stage 4. The cancer has spread to lymph nodes in the area or to other parts of the body such as lungs, head or neck. In stage 4 the size of the tumor is not important.

Recurrent cancers are those which have come back after the initial therapy is over. It may come back in its original locus, or it may come back in other parts of the body.

	How are soft tissue sarcomas 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 endocrinologist, 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 bastion of treatment for soft tissue sarcoma is surgery in which an attempt is made to take out the entire cancer along with a healthy margin of soft tissue surrounding it. Sometimes in this unfortunately, a part of an arm or leg may also have to be removed so that the cancer is taken out safely. The regional lymph nodes may also have to be removed en bloc and this is known as lymphadenectomy or lymph node dissection. 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. Radiation therapy can also be used in adjunct to surgery or chemotherapy, either before or after. 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 soft tissues 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 soft tissue sarcoma, chemotherapy is sometimes directly injected into blood vessels in the area where the cancer is found. This procedure is known as regional chemotherapy. Cancer or radiation therapy may also be used as a neoadjuvant procedure, which means that it is given prior to surgery so as to decrease the tumor in size and once the tumor volume has shrunk to manageable limits then to proceed with surgery. The purpose of this method, as it is done more and more often these days, is to prevent mutilating surgery like amputations and thus allowing a more conservative surgery to be performed. For stage 1A, 1B and 2A soft tissue sarcoma, one of the following options could be utilized:
		Radical surgery.
		Surgery followed by radiation therapy or radiation therapy followed by surgery.
		High dose radiation therapy as radical procedure followed by surgery and radiation therapy.
		If cancer is found in the head or neck or in the abdomen or chest, treatment may be one of the following options in which surgery is performed, followed possibly by radiation therapy as adjuvant or radiation therapy is given as a primary treatment and once tumor volume has gone down some form of surgery is employed.
	For stage 2B, 2C, and three adult soft tissue sarcomas, the following treatment options may be exercised:
		Radical surgery.
		Radical surgery plus adjuvant radiotherapy.
		Radiation therapy alone.
		Radiation therapy and/or chemotherapy as a neoadjuvant before surgery, possibly followed by adjuvant radiotherapy (adjuvant therapy is that therapy which is given after surgery).
	For stage 4 adult soft tissue sarcomas, the following treatment options may be exercised:
		If cancer has spread to the lymph nodes, treatment would be radical surgery to remove the tumor and en bloc dissection of the nodes to which the cancer has spread possibly followed by radiation.
		Neoadjuvant radiation followed by surgery to remove the cancer and the performance of the lymph node dissection.
		Surgery and/or radiation therapy followed by chemotherapy.
	If the cancer has spread to the lungs, treatment may be one of the following:
		Radical surgery for the primary tumor followed by radiation followed by surgery to remove the cancer from the lungs either in the form of a metastatectomy, which is just removal of the lesion or a lobectomy in which one of the lobes of the lungs are removed or a pneumonectomy in which an entire lung is removed. Everything depends on the general condition of the patient.
		Radical surgery alone.
		Radical surgery for the primary tumor followed by radiation.
		Radiation therapy followed by chemotherapy.
	If the cancer has spread to the other parts of the body, one of the following options may be exercised:
		Conservative surgery just to remove the cancer followed by radiation therapy before, or after the surgery possibly followed by chemotherapy.
		Chemotherapy just for palliation i.e. to reduce the symptomatology of the tumor and to reduce the pain and discomfort caused by the tumor.
	Recurrent adult soft tissue sarcomas. One of the following treatment options may be exercised depending upon the kind of treatment which has been offered to the patient before:
		Surgery just to remove the cancer.
		Surgery to remove the cancer followed by radiation.
		Chemotherapy alone.

What are the side effects of treatments for soft tissue sarcomas?

	Surgery for soft tissue sarcomas may leave large functional or cosmetic defects which could cause a severe functional or psychosocial burden on the patient. Hence, the role of the plastic or reconstructive surgeon is extremely important. 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.