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What is Leiomyosarcoma? Definitions LMS and Disease Progression Significance of Staging General Principles of Treatment STI-571 [Glivec] Other Treatments
What is Leiomyosarcoma?
All cancers start from one particular cell that mutates. Its DNA is damaged by mutations and changes so that the cell no longer grows in an orderly fashion according to the rules for its type. The cancer is often named for the cell type that it grows from but sometimes it has other names. Soft tissue sarcomas are cancers in which wildly growing (called malignant or cancerous) cells are from a soft tissue part of the body. The soft tissues of the body include fat, blood vessels, nerves, muscles, skin, and cartilage. Bone is not soft tissue. If the sarcoma grows from bone cells, it is called an osteosarcoma. You have essentially two kinds of muscle in your body, voluntary and involuntary. Striated [striped] muscle cells makes up the voluntary muscles; the biceps, triceps, abs, pecs...all the muscles that you can use as you wish by thinking about them. A cancer growing from this kind of cell is a rhabdomyosarcoma. Leiomyosarcoma is a cancer of smooth muscle cells. Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body: in uterus, stomach and intestines, walls of all blood vessels, skin [the muscles that give you 'goose bumps']. This muscle is involuntary muscle, you cannot make these muscles move by thinking about them. GISTs [GastroIntestinal Stromal Tumors] are a special kind of tumor, that used to be considered a kind of Leiomyosarcoma, coming from a particular smooth muscle cell in the stomach/small intestine. We mention these first and separately because there has been a dramatically good advance in treating some of these tumors with a new designer drug. See the separate page on GIST tumors and Glivec/Gleevec. Any GastroIntestinal "sarcoma" or GI LMS should be tested for CD 117 [also called Ckits] protein by an experienced laboratory. This is potentially life-saving information. Uterine LMS comes from the smooth muscle in the muscle layer of the uterus. Cutaneous LMS comes from the pilo erector muscles in the skin. GI LMS might come from smooth muscle in the GI tract or, alternatively, also from a blood vessel. At most other primary sites, retroperitoneal [in the abdomen, behind the intestines], extremity, truncal, abdominal organs and so on, the LMS probably grows from the muscle layer of a blood vessel. These blood vessels are everywhere throughout your body, so LMS COULD have a primary site ANYWHERE in your body where there is a blood vessel.
Definitions
ABBREVIATIONS are sometimes used. Here are some explanations: LMS = leiomyosarcoma GI = gastrointestinal. Includes the area from your mouth to your rectum. mets = metastases met = metastasis GIST = Gastro Intestinal Stromal Tumor. Previously thought to be a special case of GI LMS and extremely resistant to treatment. Now known to be a different kind of cancer, with a specific treatment that controls the growth. LRG = Life Raft Group. The association of people with GIST who have organized themselves into a powerful group working with the drug companies and the sarcoma oncologists in order to get more and better control of the disease for those people who Gleevec doesn't help. PRIMARY site: where the disease started with the wild division of the first mutated cell. If the PRIMARY tumor sends cells out by the blood or the lymphatic system, and those cells seed and grow, then there will be SECONDARY tumors, or METASTASIS. If the PRIMARY tumor is removed by surgery, with wide clear margins, it can still reoccur locally. This is called LOCAL RECURRENCE. Surgical EXCISION or RESECTION is removal of tumor by surgery. INOPERABLE means that surgery would probably not be able to remove the tumor mass with clear margins. EN BLOC En bloc is a French phrase meaning in one piece, it is now a standard surgical term. LMS tumors are best removed en bloc with their marginal tissue, in one large piece with the LMS tumor untouched and unseen in the center. CLEAR MARGINS When LMS tumors grow, they send out small microscopic tendrils into the surrounding tissue. If there is not a WIDE excision around the tumor, some of the tendrils will remain, and regrow later to give local recurrence. So when the primary tumor is cut out, the pathology lab must look at slices of the tumor block sections and measure the distance from the last bit of tumor to the edge of the surgical resection. The distance from the edge of the tumor to the edge of the surgical specimen is called the CLEAR MARGIN. Clear margins of 1 to 2 inches are admirable, and can often be obtained for skin or extremity [arm or leg] LMS. However, internally it can be more difficult, and a margin of 1 cm [10 mm] is wished for, 2cm would be terrific. TRUNCAL = trunk of body, essentially base of neck to groin. TUMOR WAX OR PARAFFIN BLOCK When the tumor is removed from the body, it is sent to a laboratory. In the laboratory it is put in embalming solution for some days, and then immersed in paraffin wax [like candle wax] to form a paraffin block of the tumor. The firm paraffin wax around the tumor supports it, and protects the tumor from being crushed or distorted. It allows for thin slices to be made for the microscopic examination for identification and testing of the tumor. See Testing Your Tumor. The laboratory stores the tumor wax block for years, and it is available for more tests as they become available. It might be a good idea to check with the laboratory for how long they store tumors, if some only store it for 7 years, you might wish to take it from them after a few years, as they might dispose of the tumor while you are still alive. TESTING YOUR TUMOR Testing your tumor for chemotherapy agent sensitivity, or DNA microarray may require FRESH TISSUE or FLASH FROZEN TISSUE. There are also other tests you might wish to do on the wax block tissue. See the page Testing your Tumor. SHELLING OUT This is a surgical technique where the tumor is grasped by tongs, and it is cut out along the visual margin. This can occur with LMS tumors with inexperienced or incautious surgeons. The LMS tendrils are left, and can regrow. Furthermore, interference with the tumor like this might very well increase the chance of metastases. BIOPSY There is a study done indicating that biopsies increase the chance of metastases [progression to stage IV]. If it is at all possibly, avoid biopsies. If the tumor is inoperable, you do not have a choice, really. BUt make sure FIRST that the tumor IS inoperable, by going to a sarcoma center and getting a second opinion from an experienced oncological surgeon. See Biopsies page. DE-BULKING Sometimes when tumor is large and inoperable, de-bulking--removal of a large amount of tumor without attempting to get clear margins -- is done. This can be done for comfort, but if the tumor is aggressive and high grade it often grows back very quickly. It is sometimes done in other circumstances. A PROGNOSIS is a forecast as to the probable outcome of the disease. In LMS it depends upon tumor GRADE, tumor STAGE, mitotic index, and various patient-determined properties. MITOTIC INDEX is a measure of the number of cells seen to be dividing in the microscope's high power field. Usually TEN high power fields are counted and the results added. A low count, under 10, is usually low or intermediate grade. Higher counts are high grade. Sometimes a low grade tumor will only have 1 or 2 mitoses per 10 high power fields. These tumors are often read as benign, but may not be. GRADING the tumor is determining how aggressive the tumor is. How slow- or fast- growing. It helps to suggest the prognosis. It is usually given as low, intermediate, or high grade. The mitotic index and the differenctiation are the determining factors here. WELL DIFFERENTIATED cancer cells look very much like normal cells. POORLY DIFFERENTIATED cancer cells look more disorganized and primitive than normal cells. DE-DIFFERENTIATION The process that occurs to cancer cells in the course of the disease. The cell lines in the tumor accumulate more and more mutations, and these mutations usually make the genes dysfunctional. Because of the loss of the genome protector, these mutations are passed on to the daughter cells. So the cells lose their special ordered characteristics, and become more and more disorganized and weird looking. These cells are called, at they beginning, atypical, or atypia, and as the mutations progress over months or years, poorly differentiated, then bizarre and anaplastic. As de-differentiation progresses, low grade cancers may become more aggressive. Often the intervals between recurrences gets shorter and shorter, and the tumors grow faster and faster. GENOME PROTECTOR The p53 gene on the DNA molecule is called "the protector of the genome". It prevents mutated cells who have irrepable DNA damage from reproducing. P53 protein, made by the p53 gene, has the DNA checked before it is duplicated, and again AFTER it is duplicated but before the cell splits into two. If the DNA is damaged and cannot be repaired, p53 protein starts the steps that make the cell die. This is called apoptosis. APOPTOSIS pronounced AY POP TOE SIS. This is the process by which damaged cells kill themselves, so they don't reproduce a whole line of damaged cells. "Protector of the genome" is equivalent to quality control of the DNA of the cells. The p53 gene on the DNA molecule is called "the protector of the genome" because of this. p53 damage as a cause of cancer If the p53 gene in a cell is damaged then there is no quality control on the cell's DNA --- and badly damaged DNA cells will produce daughter cells. These daughter cells will go on happily accumulating more and more mutations, until the specific mutations necessary for a cancer develop. Then it becomes a cancer cell line. p53 gene-damaged cells are difficult to kill by chemotherapy agents or radiation. Both of these techniques rely on creating gene damage in the cells, so the cell will die. But if the p53 protein doesn't work right, then the cells do not die, apoptosis does not occur, and some very damaged cells just keep on reproducing happily and bizarrely. Many LMS tumors have p53 damage, and it is this, in part, that makes them more resistant to chemotherapy and radiation. LMS tumors can RESPOND to radiation and chemotherapy, and often do, and these techniques can extend survival SIGNIFICANTLY, but radiation and chemotherapy are not usually CURATIVE for LMS. At least at this time. If radiation sensitizers or p53 genetherapy or a magic bullet is found, the situation might change drastically. There is so much research going on that it is impossible to know what will be available or in development in two years' time. NECROSIS is the death of tissue, as individual cells, or groups of cells, or in small localized areas. Necrosis AND hemorrhage in untreated tumor is considered a poorer prognostic sign because of the possibility of metastases. New necrosis in a tumor being treated with chemotherapy is a good sign, it means the tumor is responding to the chemo agent and dying. HEMORRHAGE is the escape of blood from blood vessels. If it is present with necrosis in an untreated tumor, this is considered a poorer prognostic sign. MITOSES are the visible changes in cells that are actively dividing. A tumor with many mitoses, say 30, per microscope field is likely to be faster growing than one with only 10, even though 10 is still a high number. The number of mitoses may vary from field to field in the tumor. A STAGE is a period or distinct phase in the natural course of a cancer. The disease progresses from Stage I to Stage IV. It starts small [stage I] and then grows bigger [stage II or III]. Whenever metastases occur, it is stage IV, even if the primary tumor is only at stage I or II. Sometimes stage I tumors have already metastasized, although the mets may not show up for months or years. Disease progression is the march toward stage IV [also called systemic disease]. However, local recurrences can also become problematic and inoperable and therefore life-threatening. Stage I, II, and III describe the initial tumor size and depth. A cure is possible at these stages, if the surgical excision has wide clear margins. A local recurrence does not mean that a cure is not possible. Stage I to III have different chances for cure, Stage I has the highest chance of cure, and the chances for cure are less in stage III. The chance of cure varies with primary site as well as size, so this should be looked up carefully. Mitotic index is also an indicator here too. Stage IV means there is metastasis, either to nodes or distant sites. ONE metastasis makes a stage IV. It is important to stage the disease because of the prognosis. Currently there is NO CURE for stage IV. People can live for many years if their stage IV is low grade, or if it is considerate about metastasizing to operable sites. CT scans or other appropriate surveillance are scheduled regularly to monitor the situation from the time of initial diagnosis. This is done to pick up problems early, when they are operable. However, do not be so sure that a lump picked up on scan is necessarily a metastasis. It might be a second primary cancer. 7.5% of LMS patients develop a second primary cancer. On the ACOR list there are a handful of people with 4 cancers, many of them rare. This is probably a result of an inherited or acquired gene damage, and if there is a family history of cancer, genetic counselling and testing might be available. STAGING Deciding the patient's stage of the disease is STAGING. STAGING depends upon how bit the tumor is, how deep, and how primitive looking it is. AND whether there are metastases. Staging would involve CT Scans [CAT scans] of chest, abdomen and pelvis. Sometimes an LMS tumor appearing on the skin is actually a metastasis from a primary located somewhere else. So "make sure this is the primary" is a game that has to be played sometimes. Tumors in the skin or muscle, in the lungs, in the liver and in the brain are more likely to be metastases than primaries. The importance is in the staging. There is no CURE for stage IV, even though remissions can be obtained in certain situations. REMISSION occurs when there is NED, No Evidence of Disease on CT scans or examination. No tumors seen or felt. In stage IV, LMS will usually return. Remissions extend survival time. 5 CENTIMETERS is about the same as 2 inches. LYMPH NODES are small bean-shaped organs that are found throughout the body; they produce and store infection-fighting cells. An Oncologist is a medical specialist who deals with cancer. This is a subspecialty of internal medicine. Oncologists are cancer specialists who give chemotherapy, some of them are trained to give radiation, and they refer you to other doctors for surgery or other techniques. Sarcoma oncologist are not members of a formal sub-subspecialty, but are oncologists with a special interest in sarcomas, often working at a sarcoma center. A sarcoma oncologist will probably see at least 100 sarcoma patients a year. Sarcoma oncologists will make sure that your tumor slides are double-checked by a sarcoma pathologist. Survival times are better at sarcoma centers. You should always try to get your treatment overseen by an oncologist expert in sarcomas. You do not want to be the only LMS patient in your oncologist's practice. The oncologist will not be able to put in the hours of research needed to keep updated on this rare cancer for the ONE patient in the practice. The oncologist will not have the wealth of experience of knowing the patterns of disease. You want an experienced sarcoma expert to give direction to your treatment, either directly by seeing you, or over the phone to your local oncologist. Sarcoma pathologists are pathologists [medical doctors who specialize in laboratory tests and tissue diagnosis] connected with sarcoma centers who are highly experienced in diagnosis of sarcomas. You should always try to get your tissue slides re-examined for a second opinion by a sarcoma pathologist. Surgeons have no sarcoma specialty, but the oncological surgeons at sarcoma centers will be familiar with the wide margins necessary. The first operation on a cancer can determine the outcome. An oncological surgeon will give you a better chance of survival. Radiologists -- there is no such thing as a sarcoma radiologist. But INTERVENTIONAL radiologists often do biopsies, RFA [radiofrequency ablation, see under Metastatic Disease treatments], cryoablation [see under Metastatic Disease treatment of liver mets], and some other things. Radiologists also give radiotherapy. Each specialist will recommend their specialty, if it is appropriate. An oncologist will recommend chemotherapy, a radiologist will offer radiation, a surgeon, surgery. Surgical options are the preferred treatment for LMS. See the page on General Approach to Management.
LMS and Disease Progression
This cancer will start in one place, and be very unpredictable. It can stay in one place, it can spread widely all over the body, it can spread locally or by metastasizing. It can be quiet for a long time, and then erupt after 20 years. It is a resistant cancer...not very responsive to chemotherapy or radiation. The best outcome occurs when it is taken out surgically with wide margins, early, while small and before it spreads.
Significance of Staging
Edited PDQ Statement on Adult Soft Tissue Sarcomas, Health Professional's Edition. [ [ ] indicate compiler's additional comments.] [The comments on treatment below, are generalized comments on treatments of SARCOMAS in general, NOT leiomyosarcoma in particular. This is a VERY important point, because some sarcomas are curable by radiation or chemotherapy, while leiomyosarcoma is usually not cured by these techniques. doctordee] [The treatments for LMS do differ from other sarcomas--For instance--there is NO advantage in survival time, in stage IV leiomyosarcoma, to using adriamycin and dacarbazine together. They can be used separately and sequentially for the same survival advantage, and MUCH fewer side effects. Generally, sequential treatment with single chemotherapy agents, used until further tumor growth occurs, can judiciously extend survival time in inoperable stage IV LMS. MAID is no longer frequently used. AIM [adriamycin, ifosfamide, mesna] is sometimes used to get control of aggressive cascading disease, or when a quick response is necessary for a life threatening metastasis [eg blocking off a main bronchus in the lung. doctordee] [It is for the above reason that it is important to have your treatment overseen by a sarcoma oncologist. "Sarcoma oncologists" are not members of a formal sub-subspecialty, but are oncologists with a special interest in sarcomas, often working at a sarcoma center. A sarcoma oncologist will probably see around 100 sarcoma patients a year. Sarcoma oncologists will make sure that your tumor slides are double-checked by a sarcoma pathologist. Survival times are better at sarcoma centers. See General Approach to Management Page, and references there. doctordee] [Even excellent medical facilities and cancer centers may lack a sarcoma oncologist. If you do not live near a sarcoma oncologist, find a local oncologist willing to work closely with the sarcoma oncologist. And sarcoma oncologists can do telephone consultations to local oncologists abroad as well as within the United States. doctordee] back to the PDQ statement.... The prognosis [outcome] for patients with adult soft tissue sarcomas depends on several factors, including the patient's age and the size, histologic grade[determined by the pathologist], and stage of the tumor [determined by tests, surgery, and grade of the tumor]. Patients older than 60 years of age, tumors larger than 5 centimeters, or high-grade tumor histology are associated with a poorer prognosis While low-grade tumors are usually curable by surgery alone, higher grade sarcomas (as determined by the mitotic index and the presence of hemorrhage and necrosis) are associated with higher local treatment failure rates and increased metastatic potential [tendency]. Patients with high-grade tumors (grades 3 or 4) greater than 5 centimeters in diameter have the greatest tendency for disease to metastasize and are eligible for prospective clinical trials of adjuvant chemotherapy. With distant metastases (stage IV), surgery with curative intent is possible for patients selected for optimal underlying biologic behavior (i.e., patients with a limited number of metastases, with a long disease-free interval, and with slow clinical growth) with pulmonary metastases who have undergone or are undergoing complete resection of the primary tumor. Doxorubicin alone or with dacarbazine is considered the best chemotherapeutic regimen for advanced sarcoma. A randomized trial of 340 patients with advanced sarcoma showed a higher response rate (32% versus 17%, p<.002) and longer time-to-progression (6 versus 4 months, p<.02) for doxorubicin, dacarbazine, ifosfamide, and mesna (MAID) versus doxorubicin and dacarbazine alone. The increased response rate of the MAID regimen may be justified in preoperative management of younger patients with borderline resectability, but the increased toxic effects argue against its use in older patients. Complete surgical resection is often difficult for sarcomas of the retroperitoneum due to large size before detection and anatomic location. Prospective randomized trials have not shown improved survival with preoperative or postoperative adjuvant chemotherapy for this subgroup. [HOW STAGING IS DONE] Cellular Classification Soft tissue sarcomas are classified histologically according to the soft tissue cell of origin [e.g. leiomyosarcoma, GIST,] although the cell type is not part of the staging system. Additional studies, including electron microscopy, histochemistry, flow cytometry, cytogenetics, and tissue culture studies, may allow identification of particular subtypes within the major histologic categories. The histologic grade reflects the metastatic potential of these tumors more accurately than the classic cellular classification. The histologic grade is also called the tumor grade. [Tumor Grade] Pathologists assign tumor grade based on the number of mitoses per high-powered field, presence of necrosis, cellular and nuclear morphology, and the degree of cellularity; discordance among expert pathologists can reach 40% on prospective review. [It is recommended that the slides be reviewed by a pathologist skilled in sarcomas.] Stage Information Staging has an important role in determining the most effective treatment of soft tissue sarcomas. The stage is determined by the size of the tumor, the histologic grade, and whether it has spread to lymph nodes or distant sites. Intracompartmental or extracompartmental extension of extremity sarcomas is also important for surgical decision making. For complete staging, a thorough physical examination, x-rays, laboratory studies, and careful review of all biopsy specimens (including those from the primary tumor, lymph nodes, or other suspicious lesions) are essential. Computed tomographic scan of the chest is recommended for sarcomas greater than 5 centimeters (T2) or with moderate to poor differentiation (grades 2-4). The American Joint Committee on Cancer (AJCC) has designated staging by the four criteria of tumor size, nodal status, grade, and metastasis (TNGM).2 Grade and TNM definitions
Tumor grade (G) [assigned by the pathologist. Also called histological grade.] GX: Grade cannot be assessed G1: Well differentiated G2: Moderately differentiated G3: Poorly differentiated G4: Undifferentiated Primary tumor (T) TX: Primary tumor cannot be assessed T0: No evidence of primary tumor T1: Tumor 5 cm or less in greatest dimension T1a: Superficial tumor T1b: Deep tumor T2: Tumor more than 5 cm in greatest dimension T2a: Superficial tumor T2b: Deep tumor Note: Superficial tumor is located exclusively above the superficial fascia without invasion of the fascia; deep tumor is located either exclusively beneath the superficial fascia, or superficial to the fascia with invasion of or through the fascia, or superficial and beneath the fascia. Retroperitoneal, mediastinal, and pelvic sarcomas are classified as deep tumors. Regional lymph nodes (N) NX: Regional lymph nodes cannot be assessed N0: No regional lymph node metastasis N1: Regional lymph node metastasis Distant metastasis (M) MX: Distant metastasis cannot be assessed M0: No distant metastasis M1: Distant metastasis
[STAGING OF TUMORS]
Stage IA Stage IA tumor is defined as low grade, small, superficial, and deep. G1, T1a, N0, M0 G1, T1b, N0, M0 G2, T1a, N0, M0 G2, T1b, N0, M0 Stage IB Stage IB tumor is defined as low grade, large, and superficial. G1, T2a, N0, M0 G2, T2a, N0, M0 Stage IIA Stage IIA tumor is defined as low grade, large, and deep. G1, T2b, N0, M0 G2, T2b, N0, M0 Stage IIB Stage IIB tumor is defined as high grade, small, superficial, and deep. G3, T1a, N0, M0 G3, T1b, N0, M0 G4, T1a, N0, M0 G4, T1b, N0, M0 Stage IIC Stage IIC tumor is defined as high grade, large, and superficial. G3, T2a, N0, M0 G4, T2a, N0, M0 Stage III Stage III tumor is defined as high grade, large, and deep. G3, T2b, N0, M0 G4, T2b, N0, M0 Stage IV Stage IV is defined as any metastasis to lymph nodes or distant sites. Any G, Any T, N1, M0 Any G, Any T, N0, M1
General Principles of Treatment
Remember: Leiomyosarcoma is a resistant cancer...not very responsive to chemotherapy or radiation. The best outcome occurs when it is taken out surgically with wide margins, early, while small and before it spreads. You will have an edge for survival if you are under the supervision of a sarcoma oncologist, or a sarcoma oncology center. You will hear about new developments earlier, and you will not undergo treatments of no proven benefit to LMS. TREATMENT OPTION OVERVIEW [FROM the PDQ Soft Tissue Sarcoma Patient File] There are treatments for all patients with adult soft tissue sarcoma. Three kinds of treatment are used:
Surgery (taking out the cancer in an operation) Radiation therapy (using high-dose x-rays to kill cancer cells) Chemotherapy (using drugs to kill cancer cells)
Surgery is the most common treatment of adult soft tissue sarcoma. A doctor may remove the cancer and some of the healthy tissue around the cancer. Sometimes all or part of an arm or leg may have to be removed (amputated) to make sure that all of the cancer is taken out. If cancer has spread to lymph nodes, the lymph nodes will be removed (lymph node dissection). Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external?beam radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes in the area where the cancer cells are found (internal radiation therapy). Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the blood stream, travels through the body, and kills cancer cells throughout the body. Chemotherapy that is given after surgery when no cancer cells can be seen is called adjuvant chemotherapy. In soft tissue sarcoma, chemotherapy is sometimes injected directly into the blood vessels in the area where the cancer is found. This treatment is called regional chemotherapy. Chemotherapy and/or radiation therapy may be used to shrink the cancer so it can be removed without taking off an entire arm or leg. [There are many other specific treatments available that are not the three majors listed above. The statement above is a simple one meant to help orient people. It is not a comprehensive statement. Many of the techniques and medicines and complements that can be used in certain circumstances are discussed in the Metastatic Disease section. Even this is not a comprehensive statement. doctordee] [If you are going to go on a treatment search, always look for treatment options on reputable sites, and these are NOT the sites selling the treatment. There is a lot of snake oil out there. doctordee] [If you decide to take supplements, discuss them with your oncologist. Before taking any chemotherapy or radiation or surgical treatment, tell the doctor what medicines you are taking, INCLUDING vitamins and herbals and other supplements. Some medicines, vitamins, herbals, or other supplements INTERFERE with these treatments, or cause complications. Discuss with your doctor. doctordee]
STI-571 [Glivec]
It is of overwhelming importance, if your LMS arises in the gastrointestinal tract that it be tested for cKit [CD117]. If your tumor is positive for this protein, you probably have GIST [GI Stromal Tumor] and not GI LMS. GIST used to be considered a kind of GI LMS, but actually is a different tumor. GIST was extremely unresponsive to chemo and radiation, but the presence of this protein opens up the opportunity to use a new effective designer drug treatment. The drug is known as STI-571 or Gleevec [US trade name] or Glivec [worldwide trade name]. Gleevec works in GIST because cKit is the protein driving reproduction, and throwing a monkey wrench at it stops the tumor from growing. Gleevec's possible use as an adjunctive anti-angiogenesis agent with other chemotherapy agents, or its use as a sole agent in other cKit positive sarcomas, is being investigated in various clinical trials. Gleevec is not effective in LEIOMYOSARCOMA tumors that are ckit positive, because LMS tumor reproduction is not being driven by the ckit. However, LMS tumors that are PDGFr positive might respond to Gleevec. There is just not a reliable commercial test for PDGFr at this time. Gleevec is not a drug of first choice for LMS. It is however, a drug of first choice for GIST.
Other Treatments
Currently, if the tumor can be removed completely, surgery is the treatment of choice. If cannot be completely removed, surgery and/or other methods might be used. It depends upon how many tumors, and where they are. In addition to various chemotherapy agents, radiation, and surgery.... there are radio frequency ablation (RFA), LASER treatments, anti-hormone treatments, anti-angiogenesis treatment, cryosurgery, and other specialized techniques for specific purposes. New modalities for treatment of cancers are constantly being developed. See Metastatic Disease section.

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