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General Approach to Treatment of Leiomyosarcoma
written and compiled by Roger and doctordee
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Use A Sarcoma Oncologist

First Go To Bloch Cancer Site
Then return to this page.

All LMS patients should be treated, or have their treatment overseen, by a sarcoma oncologist.
References [1,2,3,4,5]. Ammunition for Obtaining a Second Opinion at a Sarcoma Center

"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. They should see and treat sarcoma regularly - i.e. at least weekly clinics and a continuous stream of new patients referred to them, by patients, by GPs and by colleague cancer specialists. They should see at least 100 sarcoma patients a year. Sarcoma oncologists will make sure that your tumor slides are doubled checked by a sarcoma pathologist.
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.

Get all the information you can. Starting places include:
a. This website and its mirror sites.
b. Keep tuned to the ACOR L-M-Sarcoma Mailing List
Primary Tumor

LMS is a chemotherapy and radiation resistant cancer. Surgical or ablative methods are the treatments of choice. Therefore be very thoughtful if offered chemotherapy or radiation for treatment. See below.

CLEAR MARGINS are wide, cancer-free margins surrounding the tumor on the surgically removed tissue. For internal tumors, margins are hopefully 10mm or more, for external tumors margins are measured in cm or inches.

a. Definition: Adjuvant treatment is treatment given when all evidence of the cancer has been resected [removed surgically.]

b. Definition: If the surgery resulted in a total removal of the cancer, with wide, cancer-free margins on the surgical specimen, this is called surgical excision [or resection] with wide, clear margins. You need wide margins because the LMS sends tendrils out into the normal tissue, beyond the apparent border of the tumor. LMS tumors sometimes have "pseudocapsules". They look like they are encapsulated, but aren't. It isn't a capsule containing the tumor, it is tumor cells crammed up against other tissue looking like a capsule. The tumor sends tendrils beyond the apparent border of the tumor, into healthy tissue. That is why wide margins are necessary.

c. For primary tumors on many sites, adjuvant treatment has not proven to be of benefit if the surgical excision had wide, clear margins. The adjuvant treatments recommended are usually either radiation or chemotherapy, sometimes both. Either of these carries risks of creating permanent and troublesome damage to your body. Think twice about trading off the possibility of permanent damage AND loss of a treatment option, for NO proven benefit against the LMS. However, for other sites there may actually be benefit from adjuvant treatment. To be current with the latest findings, discuss decisions with a sarcoma oncologist, and look up treatment statistics about your primary site on Pubmed.

d. If you have No Evidence of Disease present [called NED, also called "in remission"] and oncologist recommends chemotherapy or radiation for you, ask for the medical article describing the research study that proves chemotherapy or radiation improves survival in your particular situation. However, if the surgical margins are positive, and not clear, often radiotherapy and/or chemotherapy can increase survival times.

e. Make sure that the research study is about LMS and NOT sarcomas in general. [Sarcomas vary in their response but LMS is a chemo and radiation resistant cancer. Surgery is the treatment of choice for LMS, followed by other ablative methods.]

POSITIVE MARGINS [Tumor cells are present at the edge or close to the edge of the removed tumor tissue.] If the surgeons feel that the margins are not wide enough, WHAT is stopping them from going in and taking more tissue out to get clear margins?

Certainly, sometimes this is done. It is much easier to re-operate to obtain clear margins on the more superficial lesions and on extremity lesions. But internally, sometimes the margins are only microscopically positive, and it cannot be determined EXACTLY where the tumor cells are--and perhaps the initial cut through them would have smeared the LMS cells over a wider surface. SOMETIMES the margins are positive because internal vital structures or organs were involved and the tumor had to be scraped off the structure or organ. Sometimes the tumor burst as it was being removed.

If tumor is left behind, often radiation [and sometimes chemotherapy] is offered as treatment, and does often confer a benefit in longer survival times. You need to see and/or discuss the research pertaining to your particular situation. Radiation in LMS is often used locally, to prevent local recurrence, or palliatively to treat inoperable tumors. Radiotherapy cannot prevent metastases.
Local Recurrence

LMS causes further problems in two ways: LOCAL RECURRENCE and METASTASIS [secondary tumors].

Local recurrence, if it is possible, is treated like the primary tumor, and is generally not considered to greatly affect survival times, unless it involves vital structures. It may or may not be followed by radiotherapy. However, repeated local recurrences might lead to locally advanced disease, which can also become inoperable.


a. Metastasis is progression of the disease, and is Stage IV. Metastases indicate systemic illness. Metastases will either be few or many.

b. If there are very few metastases, and they are operable, usually the solution is surgical resection or ablation. This may or may not be followed by chemotherapy.

c. If there are many metastases, then you are dealing with systemic disease, and systemic treatment is the treatment of choice, in order to try to get some control over the disease. Usually this is chemotherapy, but it could be targeted molecular therapy, antiangiogenesis cocktails, vaccine, or immuno- or viro- therapy. Currently, chemotherapy is the most effective method available for LMS, but those with more chemoresistant tumors should investigate other treatments.

d. LMS is a chemotherapy and radiation resistant cancer. Surgical or ablative methods are the treatments of choice. Therefore be very thoughtful if offered chemotherapy or radiation for treatment of an operable tumor.

e. Definition: Ablative methods include 'frying' tumors out of functioning existence with heat [e.g. Radio Frequency Ablation], or freezing them solid with liquid nitrogen or similarly cold substances--so that the cells burst when they defrost [Cryosurgery].

f. Consider RFA or VATS [thoracoscopic, or keyhole chest surgery] for lung metastases. If you want to know more about these techniques, ask on the LMS List, and check Treatment Techniques.

g. Consider surgery, or laparoscopic surgery, or RFA, or cryosurgery for LMS recurrences in all other locations. [LMS is a chemo and radiation resistant cancer. Surgical or ablative methods are the treatments of choice. RFA & Cryoablation]

h. Definition: Neoadjuvant Treatment. If the tumor is inoperable, then judicious use of chemotherapy or radiation sometimes actually downgrades the situation and makes it operable. The attempt to downgrade an inoperable situation into an operable one by means of radiation and/or chemotherapy is called neoadjuvant treatment.

i. Chemotherapy &/or radiation can be VERY useful, but there are good reasons to use it/them sparingly. Chemotherapy or radiation is toxic, has risks of permanent & troublesome damage, and might only control the LMS for a short period of time because resistance develops. It might be a good idea to use surgical options wherever possible, and to save your chemotherapy & radiotherapy options for controlling cascading metastases, or neoadjuvant or inoperable situations.

j. Often there is a surgical window of opportunity, which can close, sometimes within a month or two, if an LMS tumor encroaches upon, surrounds, or destroys necessary structures or vital organs. It is important not to lose this window of opportunity. For aggressive, fast-growing, high-grade tumors it is necessary to act quickly and decisively.

k. As time goes on, cancers 'dedifferentiate'. This means that they become more primitive and can act less well controlled, and more aggressive. It occurs because they are continually accumulating more and more mutations. So a previously slowly growing cancer can suddenly become aggressive and fast growing, and surprise everyone by becoming inoperable quickly. Be aware. Suddenly you don't have the time to arrange things that you thought you had from past experience, and the tumor has grown inoperable very quickly.
Test Your Tumor

Some of this is discussed in more detail in Testing Your Tumor.

a. Gleevec
If your primary tumor was abdominal, a test on the tumor wax block for the marker cKit may make you eligible for treatment with Gleevec. If you are inoperable at any stage, Gleevec is an effective oral chemostatic agent used for control of GIST [GastroIntestinal Stromal Tumors], previously considered the same as gastrointestinal LMS, now acknowledged as a separate type of tumor from GI LMS.
If your primary tumor tests positive for PDGFR, it also can make you eligible for treatment with Gleevec.

b. Markers for specific clinical trials
If your tumor is positive for EGFR, then some new anti-angiogenesis agents [eg Iressa] currently in clinical trial might help control it. There are other angiogenesis target markers as well, like VEGF. See Introduction to Angiogenesis.
Genasense, a bcl inhibitor, is in phase 1 clinical trial currently.
Alanosine is in phase 1 clinical trial currently, it requires MTAP deficient tumors.
If you are thinking of using Celebrex or Vioxx to prevent tumor growth, it would be worthwhile to see if the tumor expresses COX-2.

As more clinical trials evolve, there will be other protein markers that will help decide which tumors are more likely to respond to treatment. It is worthwhile keeping an eye on the clinical trials and discussing them with your sarcoma oncologist.

c. Genetic fingerprinting/tumor profiling [also called DNA or RNA tumor microarray]
is now being done on tumors. If you are having a tumor resected, have some of it flash frozen and stored in liquid nitrogen. This preservation can be useful for doing future DNA or other microarray on your tumor, or possibly for making a vaccine from the tumor.
There is some indication that some genetic profiling for chemotherapy agent resistance could be done on the paraffin samples, as well.

d. Chemosensitivity testing
is possible on tumors. This test requires fresh tumor tissue, which is grown in culture. Chemotherapy agents are then tried against the tumor cells. If you are having a tumor removed, it might be useful to have some sent expeditiously to the special laboratories that do this testing.
See Testing Your Tumor


Definition: Myelodysplasia is bone marrow failure, "myelo" indicates bone marrow, and "dysplasia" means that the cells are not growing correctly. Myelodysplasia can be lethal because it can cause low blood counts of one or more type of blood cells, or it can turn into cancer [often leukemia.] It is caused by multiple chemotherapy or radiation treatments damaging the bone marrow cells. Because of the very high doses of chemotherapy agents and radiation used to treat LMS, myelodysplasia is more likely to occur in LMS survivors. Development of continuously low blood counts can also prevent further treatment with chemotherapy agents or radiation.

In adults, the spine and the pelvic girdle usually contain the working bone marrow. Adjuvant radiation for uterine LMS will often create problems with the bone marrow in the pelvic girdle. Irradiation of the hip or spine likewise will cause damage to productive bone marrow.

Be aware: Ten percent of cancer deaths are a result of an increased tendency toward blood clotting. Pulmonary emboli and strokes are both lethal complications of clots.
Cancer causes a tendency toward clotting. Surgery causes a tendency toward clotting. Chemotherapy causes a tendency toward clotting. Both alcohol and caffeine act as dehydrating agents. Dehydration causes a tendency toward clotting.

Discuss aspirin, warfarin, hydration, and exercise with your doctor. Avoid long sedentary intervals [like airplane trips] without hydration and exercise. If you are having chemotherapy treatment, and cannot drink or keep liquids down and are not on an IV, contact your doctor immediately.

Stress Management

Physically dealing with the cancer is tough enough; one doesn't need additional emotional burdens that could be resolved.

Use of hypnotherapy, &/or relaxation methods, &/or psychotherapy, &/or cancer counseling, or whatever works to help deal with the often overwhelming reaction to your diagnosis, might increase survival time, but will certainly make it more enjoyable.

A diagnosis of cancer results in Post-Traumatic Stress Syndromes in a significant number of patients. It is also extremely stressful on families as well. [It is doctordee's firm opinion that every patient diagnosed with cancer should be offered psychotherapy or hypnotherapy. Psychological intervention has been shown to extend survival time in several studies.]

Caretakers are also under considerable stress. A diagnosis of cancer changes family dynamics. Psychological support or counseling should be offered to family members as well.

If your medical team is not offering professional psychological support, seek it out.

For further discussion and references, see Psychological Methods .
Alternative Medicine

We are often asked if we know of any 'alternative treatments' that 'work'.
We know of no alternative agent that will cure LMS, or bring about remission, or cause LMS tumors to undergo > 50% shrinkage. If there were an 'alternative treatment' that 'worked', we would all be on it. AND it would ALREADY have been co-opted into standard medicine.

Alternative medicine is used INSTEAD OF standard medicine. Complementary medicine is used IN ADDITION to standard medicine.

However, alternative/complementary medicine is useful in four areas. And there are many scams or interactions to beware of.

1. There are stress management and relaxation techniques. [Hypnotherapy, psychotherapy, visualization, meditation, Reiki, massage.]

2. Symptom relief.
Be careful, here. Some of the herbal mixtures to relieve chemotherapy symptoms do so by decreasing the concentration of the chemotherapy agents, and can prevent them from working to their full capacity. Some of the substances might relieve symptoms but increase metastastic potential.
Discussion with your oncologist is vital. Some over-the-counter, alternative, complementary, or herbal remedies can seriously interfere with cancer treatments. Make a list of every supplement and herbal and medicine that you take, and clear their use with your oncologist if you are undergoing cancer treatment of any kind.

3. Antiangiogenesis substances Can be used, and might have a favorable effect on slowing tumor growth. Because tumors outgrow the antiangiogenesis limitations caused by one substance, antiangiogenesis cocktails of low dose multiple substances are often used. Read about antiangiogenesis at Bill Peeples' Antiangiogenesis Site .

4. Antioxidant substances which might help prevent tumor cell dedifferentiation. [Dedifferentiation is the further accumulation of mutations by the cancer cells, resulting in more and more primitive and bizarre and possibly more aggressive tumor cells and possibly faster growing and less controllable tumors. See the discussion of dedifferentiation in Testing Your Tumor .]

5. Diet
We are often asked about diet.
See Diet .

6. Scams.
Cancer patients are often desperate. They can be easily taken advantage of by unscrupulous or well-meaning but ill-advised people. Check all proposed treatments at Quackwatch .
This is Dr. Barrett's site for evaluating questionable treatments. Even if the scam or useless agent does you no direct harm, it does take money, effort, and time from your search for more effective agents. Best to go with substances that have proven benefit.
How It Is for an LMS Survivor

If all demonstrable disease is removed, then there will be scans every 3 months for a while, then 6 monthly, then yearly. See SCANS .

Because LMS tumors do not always cause symptoms in early stages, and because they are not always accessible to physical exam, CT scans of chest, abdomen and pelvis are usually done every three months for two years, then every six months for another 3 years, then yearly. Some centers do ultrasound abdomen & pelvis and chest Xray instead of the CT scans, or alternating with them. Do not miss your scans. Unless you have cutaneous LMS, which virtually never metastasizes, you will urgently need to be under regular surveillance.

Remember also, that 7.5% of sarcoma patients get a second, DIFFERENT primary cancer. Surveillance can often detect these cancers as well.
Fetch PMID: 11283938

There may never be another episode of LMS.
OR there might be a local recurrence, or a distant metastasis.

If LMS reappears, the treatment of choice is surgical. If the tumors are resected [surgically removed] or ablated, then the patient is again in remission.

If at any time, the tumors cannot be surgically resected or ablated, then there is use of chemo and/or radiation, hoping to knock the tumor back enough to MAKE it surgically resectable [this is called neoadjuvant treatment].

If many metastases occur, or cascades of metastases occur, chemotherapy is usually used to try to control the systemic disease. Chemotherapy and radiation do not cure LMS, but sometimes they work well enough for long enough to allow a surgical solution or control where none existed before. There is a slight chance that chemotherapy or radiation might eradicate tumors without the necessity of surgical excision, and put the patient back into remission.

If at any point LMS tumors cannot be either ablated or resected surgically, then palliative treatment is given, usually either with radiation or chemotherapy, in an effort to prolong survival time. There is still a slight chance that chemotherapy or radiation might eradicate the tumors.

There are other treatments becoming available that might be used in addition to chemotherapy, or in an adjuvant situation. Among these are Gleevec for PDGFr positive tumors, anti-hormone treatment for tumors with hormone receptors, antibody targeted viruses for tumors, replacement p53 gene therapy, vaccines, monoclonal antibodies, anti-angiogenesis treatment with targeted molecules or copper-lowering agents or metronomic dosing, targeted molecular tumoristatic agents, -- the list goes on and on. Some of these are discussed more fully on this website, for others you will have to use PubMed and ASCO searches, or find other sites. You can also join and ask on the L-M-Sarcoma mailing list Click Here

Darcey's three rules:
Truthfully, there are only three pieces of advice any one of us needs to receive.
a. Get competent medical care our case a sarcoma expert
b. Question everything and listen closely to what others are trying. You never know when you will need it
c. If a Dr tells you 'no' about a certain treatment, keep knocking on doors until someone says yes...even if they never do, you will know you have tried.
Clinical Trials

Participation in clinical trials for inoperable LMS stage IV is often urged because of the scarcity of effective nonsurgical treatments. Be aware of and read about the Clinical Trials being run.

Search Soft Tissue Sarcoma and Uterine Sarcoma categories.
PDQ search at

A Google Search sometimes turns up Clinical Trials, and sites that list clinical trials. CLINICAL TRIALS information is also available on other sites as well.
Ammunition References

When dealing with HMOs or medical insurance companies or government institutions [VA hospitals, Army, Navy, Air Force] you may need ammunition for justification to get sarcoma oncologist treatment at a major sarcoma center.
Go to the original online sources listed below and print them out. Using the printout to justify your intended treatment is a perfectly acceptable use of copyright material. You will not get into trouble.

These articles recommend the use of sarcoma oncologists and sarcoma teams at sarcoma centers to manage sarcomas, because patient survival is so much better.

1. The October, 2000 issue of the Lancet Oncology Journal states, "When diagnosed and managed in a non-specialist environment, outcome is generally significantly poorer than if patients are managed by a multidisciplinary team in a tertiary centre of excellence. Prompt referral of patients with clinically suspicious masses is strongly advocated, before any type of intervention is attempted."
The full article is at the Lancet Oncology Journal. Complete the free registration, then search for Singer, Demetri,, "Management of Soft Tissue Sarcomas: an Overview and Update", Lancet Oncology, October 2000.
[You may have to order the full text from your local librarian.]

2. Further justification for using a sarcoma oncologist and a sarcoma team at a sarcoma center to manage sarcomas is given by the NCCN guidelines. These two references, the Lancet Oncology article and the NCCN guidelines are useful when petitioning insurance companies or HMO's for reimbursement for sarcoma oncologist referrals.

The NCCN guidelines repeatedly state: "All patients should be managed by a multidisciplinary team with expertise in sarcoma."

NCCN Guidelines for Soft Tissue Sarcomas and for Uterine Sarcomas can be obtained from the NCCN, or downloaded from their site. NCCN site Choose the Health Professional versions, always.

National Comprehensive Cancer Network
50 Huntingdon Pike, Suite 200 Rockledge, PA 19046 Phone: 215-728-4788 Fax: 215-728-5641 ; 215-728-3877
You have to state they are for your own use and for information purposes only. They are free. You do not have to be a doctor.

Use of NCCN guidelines to convince someone at your HMO or medical insurance agency of the necessity of sarcoma oncologists is a perfectly valid use of them. These are GUIDELINES. For guidelines to function, they have to be disseminated.

3. Eur J Cancer 1996 Feb;32A(2):269-73
The importance of a multidisciplinary group in the treatment of soft tissue sarcomas.
Wiklund T, Huuhtanen R, Blomqvist C, Tukiainen E, Virolainen M, Virkkunen P, Asko-Seljavaara S, Bjorkenheim JM, Elomaa I.
Department of Radiotherapy and Oncology, Helsinki University Hospital, Finland.

"In 1987, a multidisciplinary soft tissue sarcoma (STS) group was established and a treatment protocol was set up. By 1993, there were 193 patients with a diagnosis of STS of the superficial trunk or extremities. ...."
"The estimated 3-year survival, local control and disease-free survival rates are 79, 87 and 69%, respectively. These results compare favourably with previously published results from this hospital and from a nationwide study. The improved results emphasise the importance of a multidisciplinary STS group."
Fetch PMID: 8664040

4. 2002 CTOS Annual Meeting Oral Presentations - Surgery
Copyright 2003 Connective Tissue Oncology Society

[Abstract ID: 48]
Robert Grimer, Aneel Bhangu, John Beard; Royal Orthopaedic Hospital, United Kingdom

"Study Question: Whether patients with soft tissue sarcomas do better if treated in a specialist center compared with district general hospitals."

"All soft tissue sarcoma patients' outcomes were analyzed in a geographical health region in the UK for three years, with at least a 5 year followup period. Appropriateness of treatment, adequacy of surgery, and outcomes in terms of local control and overall survival were investigated. Results are stratified for known risk factors for local control and survival (grade, depth and size)."

"260 patients were diagnosed as having STS over the 3 year period of whom 37% had the majority of treatment at the specialist centre under the care of 2 surgeons, whilst the other 63% were treated at a total of 38 different hospitals. Local recurrence rates were 20% at the specialist centre and 37% at the general hospitals. Overall survival was 58% at five years and was related to grade, depth and size of tumour. Patients treated at the specialist centre had larger tumours (10.3 vs 7.3cm) with a higher proportion of deep and high grade tumours. Overall survival at the two centres was identical but when stratified for known risk factors the survival rate was 1.6 times better at the specialist centre, this difference being especially obvious for Stage III tumours (p = 0.009). "

"Conclusions: Soft tissue sarcomas are rare. Centralization of treatment, especially for high grade tumours improves survival, local control and patients' care."
Fetch Abstract

5. Ned Tijdschr Geneeskd 1995 Apr 22;139(16):833-7
[Consensus soft tissue tumors. Dutch Workgroup Soft-Tissue Tumors]. [Article in Dutch]

Van Geel AN, Van Unnik JA, Keus RB. Dr. Daniel den Hoed Kliniek, afd. Chirurgische Oncologie, Rotterdam.

"Soft-tissue sarcomas constitute a rare group of malignant tumours with histopathological features of connective, muscular, fatty or peripheral nervous tissue. The prognosis at manifestation depends on only two factors: the spread, both local and remote, and the biological behaviour of the tumour. The latter factor cannot be influenced but the former can: by inexpert manipulation. Consequently, tumours suspected of being soft-tissue sarcomas require multidisciplinary management from the beginning, with the team members familiar with each other's diagnostic and therapeutic skills. Imaging diagnostic methods should precede invasive methods for collection of material for pathological examination. The number of mitotic figures observed at microscopical examination of the tissue is an important prognostic feature. Surgical resection is the treatment of first choice. Radiotherapy is indicated in grade 3 tumours, after recurrence surgery, and when radical resection would involve too much mutilation. Chemotherapy is only given in the context of clinical trials. Surgical treatment of lung metastases may be indicated in selected patients. Regional isolated perfusion with tumour necrosis factor may be an alternative for limb amputation. Publication Types: Consensus development conference Review "
Fetch PMID: 7731476

6. Sarcoma, Vol 7, Nos. 3-4/September-December 2003 pp159 - 165

Factors influencing prognosis after initial inadequate excision (IIE) for soft tissue sarcoma

Albert N. Van Geel, Alexander M.M. Eggermont, Patrick E.J. Hanssens, Paul I.M. Schmitz
University Hospital Rotterdam/Daniel den Hoed Cancer Center Rotterdam The Netherlands

"The influence of initial inadequate excision (IIE) of soft tissue sarcoma (STS) on local control and overall survival is not well established. ...."

"Specimens of the subsequent radical resection showed residual tumor in 66 patients (77%). In ... analyses, grade II/III tumors and complications after IIE are significant negative prognostic factors for local recurrence-free survival (P = 0.008 and P = 0.002, respectively, in the Cox model). ... Adjuvant radiotherapy did not change the rate of local recurrence-free survival. For overall survival, only tumor grade is a significant factor (log-rank test)."

"... For better diagnosis and therapy STS should be treated in specialized centers."
Fetch Abstract

written by doctordee, last updated January 2004

"How long have I got, doc?"
Written by Roger, updated November 2002

For some people this is their first thought when diagnosed with cancer, whether the question is actually asked or not. No one can answer it with any accuracy for leiomyosarcoma.

This page may not make easy reading. We have tried not to 'pull any punches' - which means that what we say may not be what you had hoped to hear. Always remember that every case is different and that this is general information, intended to help you frame questions for your doctors to answer. Only their opinion is valid for you.


Doctors usually quote a statistical chance of surviving cancer five years from diagnosis expressed in percentages. Statistics take on the meaning you want them to. When you are upset and uncertain after a diagnosis, a misunderstood statistic can take on a disproportionate importance. If you are reading on, read with this in mind.

The initial prognosis offered by a doctor will be drawn from five factors: the size, location, and grade of the tumor, plus the 'staging' of the disease, and whether the cancer was completely removed with wide, clear margins.

The critical aspect of size is that if the primary is under 5cm, history shows average survival to be longer. Small tumors, expertly excised, have the highest likelihood of offering long term remission - the closest term anyone will get to 'a cure.' On the other hand, large tumors are more likely to be associated with metastasis at the time of diagnosis.

Location has multiple repercussions. An extremity tumor (leg or arm) is likely to be seen and thus to be found when smaller. Such a tumor is unlikely to impact on other organs and obtaining 'clear margins' in surgery is easier. An internal tumor will remain hidden for longer, requires more complex surgery, and getting clear margins may impact on other organs.

There are various systems for grading a tumor. However the grade of most LMS is usually 'high' whatever system is used. Some LMS is 'low' grade but that is unusual. The grade describes the activity of the cancerous tissue, and thus its readiness to reproduce.

Staging is a way of describing how widespread the disease has become. A low grade extremity tumor, even with local recurrence, will be Stage 1. If there is distant metastasis to the lungs or liver, the disease will be in Stage 4B, even if the tumor is low grade and the disease has only just been diagnosed.

The honest doctor will add that no one knows when, or in which patients, or why, LMS recurs. There is evidence from unpublished data that surgery at a non-specialist hospital increases the probability of local recurrence. Statistics on local recurrence also show that there is a higher likelihood of recurrence from a retroperitoneal primary (growing from the abdominal or pelvic wall) than from an extremity primary tumor. LMS is aggressive and tumors can grow very large, very quickly.

Local recurrence is not so bad for prognosis as metastasis to a distant part of the body, or to the lymph system.
Abdominal tumors have a greater likelihood of metastasizing to the liver while extremity tumors and uterine tumors are more likely to metastasize to the lungs.

Clearly, as disease becomes more advanced the odds against longterm survival increase, but the statistics are far from being a perfect indicator. The spread of LMS can be very individual, and responses to the different kinds of treatment vary greatly.

The statistics for LMS are also badly affected by the historical inclusion of patients with GastroIntestinal Stromal Tumor (GIST) in most of the research. Separating statistics for the two is difficult. Taken all together, survival to five years after diagnosis is achieved by about 40% of patients with high grade tumors, and 70% with low grade. As GIST was almost impossible to treat effectively until the recent development of Gleevec, these average figures are set to improve.

Some other factors which can affect a prognosis:

Lymph node metastasis (Stage 4A) occurs in only about 3% of cases. It can usually be excised surgically and general prognosis will be better than for a patient at Stage 4B.

Response to chemotherapy varies widely. Approximately 20% of patients treated with chemo for an active tumor demonstrate a good response. The median time for further recurrence is about 15 weeks (this means that half of all patients achieving a response show new tumor growth within 3 to 4 months).

Initial remission (i.e. remaining clear of disease after surgery to remove primary tumor and with no initial evidence of spread of the disease) can extend to many years and the longer the time achieved the longer remission is likely to continue.
Long term remission (i.e. more than a year) after distant metastasis is rare but there are documented cases.

But even when long term remission doesn't occur, long term survival with active disease is still possible. There are known cases where a patient has lived an active life for several years, although this has often involved successive treatments to gain short periods of remission.

November 2002

And some people NEVER see LMS again in their lives, despite everything.

REMEMBER: Statistics just indicate that you have a serious situation. They do not apply to individuals, ONLY to groups.

The percentage of LMS tumors that recur or metastasize depend upon tumor site, size, grade, stage, presence of necrosis and hemorrhage, biopsy, tumor-positive margins, p53 gene damage, and, for ULMS, mitotic index. Simply, stage and grade are the two most important prognostic factors for recurrence/metastasis.

[However, another criterion is important for survival, and that is operability. The luck that metastases or recurrences that do occur, can be removed surgically or ablated. Metastasectomies can considerably increase survival time.]

To find out YOUR percentages for your site and particular tumor properties, you can:
  1. Ask your sarcoma oncologist.

  2. See Primary Site Statistics in this section.

  3. Wendy, an ACOR L-M-Sarcoma List Manager, has the algorithm program from MSKCC that will give death rates at 12 years for specific primary sites and grades of LMS tumor. Ask on the L-M-Sarcoma Mailing List at ACOR, or look up the algorithm program online on the MSKCC site and download it.

  4. Do your own Pubmed search for your own primary site. Go to Pubmed and type in the search box "survival AND XXXXX leiomyosarcoma" [but without the quotes.] But put the name of your primary site there instead of XXXXX, then click on GO.

  5. And having done all that and read all the statistics.... still... Statistics just indicate that you have a serious situation. They do NOT apply to individuals, ONLY to groups. And having stage IV LMS, while it is not currently curable, does not mean imminent demise. Some LMS patients have been in stage IV for 12 years and more.
Rhythms of Treatment

For those people who are in remission [NED -- No Evidence of Disease]
The rhythm is scans every three [or six or 12 months.]
Life is lived and appointments and plans are made up to the scan date, but not for afterwards, until the scan results are known.
I think though, at 5 years and yearly scans... people are more relaxed because there is less worry about sudden inoperable disease appearing.

For those who are being treated with chemotherapy
There is the starting of the chemotherapy treatment.
Then the rhythm of the chemo takes over. First week symptoms, second week fatigue, third week feeling better, then the cycle starts again, with scans every three cycles or so.
And there is a comfort when one is on chemo.
One is doing all one can at the time.
One feels that one doesn't have to think or plan much beyond the next cycle.

However, if the chemo ends or isn't useful? What to do then? Especially if the scans have shown growth?
The question of what to do next should be already answered.
Get the plans in order for the next chemotherapy while the tumor is still responding to the current chemotherapy regimen.
Have the tumor tested for specific proteins if need be. Contact the doctors for the trials. Get everything lined up just in case. Do not wait until there is a scan showing growth to start investigating the next options. Get things set up so that the next choice of trial or chemo can be started as soon as the current chemo is ineffective.

Usually scans and blood tests are done at the end of a three or four week interval just before the next chemotherapy infusion. If the scans show growth, and if the patient is already set up with another doctor or trial, these scans and blood tests can be used for the baseline for the next chemo. And the results can be sent immediately to the doctors so the next treatment could be started within the week. Avoid waiting another four or more weeks in order to investigate the next step.

January 2004

The information on this site is not a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with your doctor. Please consult your doctor with any questions or concerns you may have regarding your condition. Copyright 2001-2010 LMSWEBSITE