|First Steps After Diagnosis
written and compiled by doctordee
|Search LMS site|
The newly diagnosed patient is often dazed, in shock, anxious or worried or confused. They often get nightmares, or can't sleep...or feel like sleeping all the time.
"I functioned in a kind of daze for at least 6 weeks. I mean...I could do things and respond and looked normal and all that... but I was in never-never land inside my head." [doctordee]
As you understand more, you can make informed decisions, and the worry and anxiety and shock do go away as you re-establish your control of your life. Knowledge and perspective make a big difference. It's a lot of work, but it is do-able.
This is a time when some people will just continue with business as usual. They go for whatever medical or surgical treatment is necessary, but ignore the fact that they have cancer, and any of the implications of having cancer.
This is a time when some people will do displacement activity...ANYTHING other than try to deal with having cancer. Houses get cleaned from top to bottom. The neighborhood gets put under control. Afghanistan gets reorganized. There are major efforts made for World Peace.
The choice to deal with what you've got, what you need to do, and try understand it all is a very important one.
We request that you now proceed to the Bloch Site. This is an attitude determining site. Read the introductory letter, and either print the book, or call the telephone number and have the book sent to you free of charge.
When you have finished with the Bloch Site, return here for further directions on what you need to do.
|Do I [Really] Have LMS?|
"Right now, I'm having a day-to-day battle with my brain. I have cancer...do I really have cancer? Oh yes, that's right, I have cancer. What do I do now?." [Erica]
"Are you sure I have cancer" is pertinent, a lulu, and gets right to the heart of the matter. An even better question is "Are you sure I have leiomyosarcoma?". Leiomyosarcomas are difficult to treat, it might be a different cancer, one that is easier to treat.
Leiomyosarcoma is extremely rare. Some pathologists may never have seen one before. There are, however, pathologists who have a major interest in sarcomas, and who review them all the time. Your tumor tissue should be examined by a sarcoma pathologist for confirmation.
And because leiomyosarcomas are rare, difficult to treat, and don't react even like other sarcomas, it would be best to go to an oncologist [cancer doctor] who has a major interest in sarcomas, and treats a lot of patients with them. We call these doctors, informally, sarcoma oncologists. They usually practice at a sarcoma center, or a cancer center of some kind.
Sarcoma oncologists recognize that leiomyosarcomas do not respond to some treatments that other sarcomas do respond to. Where a general oncologist might treat you just like any other soft-tissue sarcoma, there are some situations where the treatments may not give any proven benefit, and do give damage. There are also some situations where there have been new developments which are very exciting, but require the tumors to be tested.
One of these situations is adjuvant treatment of a truncal LMS that has been removed with clear margins. Another is the development of STI-571, a new designer drug, which works phenomenally well in clinical trials on GIST LMS. We will discuss these further on.
The "Doctors" section can help you with finding a sarcoma specialist, so can the LMS List at ACOR.org. See the section on Resources.
After choosing him/her, you might call up the sarcoma oncologist's office and make a tentative appointment. Make a list of what is required....tissue samples, addresses, reports, referrals.
Go to your primary doctor. State to him/her: I have a rare cancer. I have heard that this doctor [the sarcoma oncologist] treats a lot of these particular types of rare cancers. I would like a referral to this doctor for decisions about treatment and management of my condition. [You are entitled to a second medical opinion by an expert. See Patients. Rights section.]
Now if you live pretty far from the sarcoma oncologist, the sarcoma oncologist might be making decisions for treatment with you, that your oncologist nearer home will be carrying out.
Go to your general oncologist [if you have been referred to one] and state: The sarcoma oncologist treats a lot of leiomyosarcomas. I am being referred to him for second opinions on treatment options. Do you have any objections to this? Would you be willing to carry out some treatments recommended by the sarcoma oncologist, here, nearer my home, if it were possible?
You can also see another sarcoma oncologist, should things not jell well with the one you are seeing. Your choice of doctors is very important. And you can change doctors if you think that your needs would be better met by another doctor. We would especially recommend that you change doctors if your doctor is not interested, encouraging, helpful and hopeful.
|Get Your Records|
"I've been sleeping better since I decided to get a second opinion, but there's so much info to absorb, I'm feeling rather overwhelmed. I'm sure that's normal, but somehow that doesn't make it an easier or make me feel any better." [Erica]
The next things you do are ask for your copy of the pathology report and the operation report, and any correspondence between the doctors about your case.
You probably have had a spiral CT scan of the chest, abdomen and pelvis with and without contrast. You will need that report.
You will need reports of any laboratory test results, or other XRays or tests.
You will also need a case summary of your whole medical history, not just the cancer. This is needed for a referral letter, anyway, and your primary care physician/GP should be able to provide one.
[You are entitled to all of this information. See Patients' Rights section.]
Take the pathology/histology report and the operation report and the CT scan report. These are probably the most important in terms of understanding the cancer. But in order to understand these reports, you will have to understand a LOT more about the disease, and how it works, and what significance the words have.
You will have to translate the operation report and the pathology report into English from Medical Jargon. You can go to the library or your doctor's office and use a medical dictionary, have the surgeon or pathologist or your GP explain it to you, word for word, or have a friend in the medical field help you out. In any case, write the English translation down separately.
There is a MOUNTAIN of information to understand. But you can do it. It just seems a lot... in a few weeks, you'll be able to teach other people about it [likely including your GP].
This is not a TV serial where everything is resolved within an hour. It will take some time. The important thing is that you are facing it... not hiding away. Doesn't mean that you have to live with it every minute of the day, though. And being a cancer patient doesn't mean you're going to die from it. Knowledge and perspective make a big difference.
Next Step... Figuring out WHERE you are and WHAT choices you have.
|What to Do Next|
Arrange for a referral to a sarcoma oncologist.
Arrange for tissue and slides to be sent there for review by a sarcoma pathologist.
Arrange for testing of the tumor for CD117, PDGFr, and, if uterine, for estrogen and progestogen receptors.
Send or take copies of the pathology report, operation report, any other laboratory results.
Send or take a summary of your case history [past medical history included].
|WHERE are you, in the staging of the illness?|
Where you ARE, determines your choices of treatment, and your need for treatment.
You will still need close medical follow-up every 3 months.
ISOLATED PRIMARY, SURGICALLY RESECTED, margins NOT clear. This means that you may very well have cancer left behind.
Further treatment is usually done, either surgical if possible, or radiotherapy or chemotherapy.
PRIMARY, SURGICALLY RESECTED WITH CLEAR MARGINS, but METASTASES [other tumor(s) present in lymph node or distant site.]
Further treatment to deal with the metastases will be necessary.
PRIMARY, SURGICALLY RESECTED, margins NOT clear, and METASTASES.
Further treatment will be necessary.
LOCAL RECURRENCE only.
You will need treatment.
|LMS Primary or Metastasis?|
Make sure you know where the primary tumor is. The tumor in the lung, bone, limb, skin, could be a metastasis from a primary in another site.
The recognized primaries are uterine, retroperitoneal, gastrointestinal, great vessels. Tumors of these sites are very unlikely to be metastases from another site...although uterine primaries can seed throughout the abdominal cavity as well.
Uterine leiomyosarcoma is often hidden within 'benign' uterine fibroids. Sometimes the pathologist reporting on the hysterectomy specimens misses the LMS cells hidden away within. The lung tumor that may appear years later is then a metastasis, and not a lung primary.
Knowing that the lung tumor is a metastasis is not only important for the staging of the LMS, but for redirecting attention to possible local recurrence that was missed in the pelvic or abdominal cavities.
Lungs are classic sites of metastatic tumor. They are also capable of sending out metastases as well, to limbs, skin, bone...in fact to every organ or system that has a direct blood supply.
Livers are also classic sites of metastatic tumor. Abdominal tumor tends to metastasize to liver, but so can lung. However, there has also been reported a case of LMS with a primary tumor in the liver.
LMS in the heart, or adrenal gland, or thyroid gland, or bone, or muscle could be primary or metastatic. There are very few sites which have never been reported as a primary LMS, and very few sites which have never been reported as metastatic.
Leiomyosarcoma does not primarily metastasize by lymphatic spread, but it can and does spread to lymph nodes. Regional lymph node involvement is more common in pelvic and abdominal LMS, where it can occur in up to 14% of the cases, but can also occur in other sites, as well. But there was also a case reported of an LMS primary in a lymph node.
So a thoughtful and responsible approach to the diagnosis of LMS in ANY site, is a thorough examination and a Spiral CT Scan of Chest, Abdomen, Pelvis with and without contrast, looking for more tumors in other places.
written by doctordee April 2001
|Nature of Leiomyosarcomas|
Leiomyosarcomas are well known for local recurrence and metastatic spread. The metastases are usually [95% or more] spread through the bloodstream, but there are cases of lymphatic spread.
Early surgical removal of the tumor, before it spreads elsewhere, and with wide clear margins, so it is less likely to recur in the same place, is the best thing to have done, and this MIGHT accomplish a cure.
Tumor invasion of lymph channels or blood vessels, or of other organs, larger size of tumor, necrotic areas with hemorrhage, many mitoses...all are poorer outcome signs, because of the likelihood that the tumor is more aggressive and has already metastasized.
Because of the "returning" nature of the LMS, it is still necessary to have close supervision by a cancer doctor every three months. If metastasis or local recurrence occurs, it can be dealt with. Surgical intervention is generally considered best if it is possible, unless the tumor is CD117 positive and the designer drug, the chemostasis agent STI-571 [Glivec] can be used. Then you may have choices.
|General Principles of Treatment|
A sarcoma oncologist will generally give you an edge. Frank discussion of effectiveness of adjuvant [see next section] or other therapies is very important. Except for those which are CD117 positive and respond to Glivec, the tumors' response rate to chemotherapy or radiation treatment is not high. The success rate is on the order of 1/3 of the tumors showing response...the cure rate, however is very, very low. However, these drugs can, if successful, buy you time. Because none of the chemotoxic agents are very effective with LMS, there are new drugs being tested all the time.
Unless a tumor is growing in a sensitive place, it is often not the local recurrence which is eventually fatal, but the metastases--either by bulk, or by other effects. Chemotherapy or radiation treatment can keep the bulk and effects of the metastases under control, but at the expense of your bone marrow and/or liver.
Furthermore, radiation and chemotherapy have some permanent effects on bone marrow. Chemotherapy can also injure liver. Too many treatments, and your bone marrow no longer makes blood cells [see Myelodysplasia (My-ehh-low dis-play-zya)], or your liver no longer works. At these points, the oncologists will no longer give you chemotherapy or radiation.
This is another reason why surgical resection is the management of choice, if it is possible.
For more information on the approach to treatment of leiomyosarcoma,
The General Approach To Treatment of LMS
|LMS & Treatment STI-571 & CD117 & GIST|
The experimental drug STI-571, now known as Glivec, is undergoing clinical trials against some forms of Leiomyosarcoma.
It appears to be effective against LMS tumors that have a certain protein/enzyme active in their metabolism. Testing of the tumors for the protein CD117 [or c-kit] is necessary. If the tumors are c-kit positive, [CD117 positive] then approximately 80% to 90% of those tumors will respond to treatment with STI-571 [Glivec], and start shrinking.
Glivec is a designer drug, a Signal Transduction Inhibitor, not a general chemotherapy drug. This means that the side effect profile is much milder than for the usual chemotherapy agents.
Because of the early but VERY impressive response to STI-571, we urge ALL abdominal tumors to be tested for CD117 [c-kit]. Because the CD117 [ c-kit] is sometimes, though rarely, expressed in LMS tumors which are not gastrointestinal, it might be a good idea to test every other patient as well.
STI-571 (Glivec) has now been successfully used in clinical trials against chronic myelogenous leukemia (which expresses bcr-abl) and against GIST (which expresses c-kit). There is some semantic and diagnostic confusion about the definition of GIST and whether c-kit positive tumors outside of the gastrointestinal area are technically GIST.
There are a few other cancers which may also demonstrate c-kit. The most likely one, for which Novartis is planning clinical trials, is small cell lung cancer. This is quite different than large cell lung cancer, the more common disease.
There is also a possibility that STI-571 may work against cancers which demonstrate platelet derived growth factor (PDGFr). The most likely one, for which Novartis is planning clinical trials, is prostate cancer.
STI571 is a "designer" drug which targets a discreet number of cancers.
Tests for c-kit, or for anything else, are sometimes wrong. When confronted with conflicting results from two major medical centers, one may want to consider a third. It is hard to say which one of the first two was correct.
|FAQ About GIST and C-kit and STI-571|
Does STI-571 work against all GIST tumors?
No. In early Clinical Trials, it seems to be effective in shrinking tumors between 80% to 90% of the time.
How fast does STI-571 work against GIST tumors?
Generally, significant shrinkage is being seen within 30 days.
What is the c-kit test?
It is a test for CD117, or c-kit, a protein on the surface of the cell membrane.
What is GIST?
A gastrointestinal primary tumor, Gastro Intestinal Stromal Tumor, considered LMS, but descended from a slightly different smooth muscle cell. All leiomyosarcomas show markers for smooth muscle (ie actin), but GIST arises from a specialized smooth muscle cell called Interstitial Cell of Cajal. If you have GIST, find out if you are c-kit positive. If so, you are a candidate for STI-571 should the need arise.
How do I know if I am c-kit positive?
A slide from your tumor is stained/tested and looked at under the microscope by a pathologist.
Does it matter if the tumor slide is from my primary tumor or from one of my mets (secondary tumor)?
No. But it needs to be a good sample of the tumor, not from an area that is necrotic.
How do I get my tumor tested?
The original laboratory that reported on your LMS will have the tumor kept there. They can prepare new slides for a test. It is suggested that you have the test done at a laboratory that does a lot of c-kit tests...like a sarcoma center laboratory.
Is c-kit [CD117] the only indication that STI-571 might work?
A tumor needs to be positive for c-kit, OR Abl, since these are the known targets of STI-571. Abl is related to the Philadelphia chromosome, and is not relevant to LMS. The PDGFr [platelet derived growth factor] is another test that can be done on your tumor for possible indication of a trial of STI-571.
Is the c-kits test 100% reliable?
No. No test is 100% reliable and c-kit test results can sometimes be false positive or false negative.
If my primary tumor was not in the gastrointestinal area (e.g. a uterine tumor), can it still be c-kit positive?Anything is possible, but that is unlikely.
What does it mean, then, if a uterine primary tests c-kit positive?
It means that it is probably a false positive. It can be re-tested.
Will STI-571 work against leiomyosarcomas that are not c-kit positive?
Probably not, but the European trials will be testing that by offering STI-571 to a small number of c-kit negative sarcoma patients.
Is STI-571 a type of chemotherapy?
No, it is not. Chemotherapy drugs are cytotoxics and they kill dividing cells with little regard for whether or not they are cancerous. STI-571, or Glivec, is a member of a new class of drugs called cytostatics--they are designed to home in on cancer cells without harming healthy ones.
Does that mean that the side effects of STI-571 are less than those of traditional chemotherapy?
Generally yes--a lot less.
My doctor knows that I am c-kit positive but wants to do surgery anyway. What should I do?
Unless you are in a life threatening situation, you should consult a sarcoma specialist about STI-571 as an alternative.
My doctor knows that I have GIST (i.e., am c-kit positive) but wants to treat me with traditional chemotherapy anyway. What should I do?
Get another doctor, preferably a sarcoma oncologist specialist.
Should I trust the information above?
You should always do your own research and consult with your own physician.
The author of this question and answer series is not a physician and is simply an informed lay person trying to share his understanding of a complicated area. In doing so, the answers tend to be somewhat simplistic and may not always be completely accurate. In addition, keep in mind that the STI-571--GIST trials are very recent and still involve small numbers of patients. The author only has access to the early trial results of a small subset of trial patients who are members of the Life Raft Group--they may or may not be representative of the trial group at large.
Where can I get more information?
See the Article-Jerry & Norman at: http://www.acor.org:/lrg/
|Adjuvant Treatment OR Not?|
For the situation of:
ISOLATED PRIMARY, SURGICALLY RESECTED WITH CLEAR MARGINS.
Sounds like it was completely removed at this time... so there isn't any left, is there? So you may be a 'cancer patient' but you may not have cancer!!!!
Any treatment done at this time is called "Adjuvant therapy" or "assisting treatment". The first treatment, the surgery, removed all visible trace of cancer from your body. The "assisting" or "adjuvant" treatment of chemotherapy or radiation therapy is meant to help remove even microscopic traces. However, for LMS, in some situations, this adjuvant treatment does not give any proven benefit.
Many sarcoma oncologists would recommend waiting until there was something to treat.
Your body can only take so many treatments of radiation and/or chemotherapy. If you use up part of this allowance, for no proven benefit, you may not be able to have treatment should you have large tumors that need it, later on.
Some sarcomas do respond to adjuvant therapy. However, often in certain locations, LMS does not respond to either radiation or chemotherapy as adjuvant therapy.
It is wise to discuss this thoroughly with your sarcoma oncologist.
At this point, people are usually desperate to do something that will kill all possible LMS, whether it is there, or not. They will undergo chemotherapy, they will undergo radiation. However, sometimes this chemotherapy or radiation will have no proven benefit.
Do not let your desperation to be free of cancer cause you unnecessary physical damage. Damage for no proven benefit.
Neither radiation nor chemotherapy are benign treatments. They cause damage to your body. Some of this damage is permanent. Some of this damage may even be fatal.
This is a decision that requires careful thought, and reading of the research literature. And further discussion with your oncologist.
Do not act hastily. Think. Discuss. Read.
|PDQ files and the NCI|
The National Cancer Institute [NCI]
The National Cancer Institute (NCI) is the lead Federal agency for cancer research. Since Congress passed the National Cancer Act in 1971, NCI has continued to collaborate with top researchers and medical facilities across the country to conduct innovative research leading to progress in cancer prevention, detection, diagnosis and treatment.
CancerNet is part of the NCI, and provides information, a dictionary of Cancer terms,the CancerLit search engineand PDQ files on treatment and Clinical trials. Browsing through the information offered on this site is well worth it. Nothing on this site is classified or restricted or under copyright.
Physician Data Query database [PDQ]
PDQ - NCI's Comprehensive Cancer Database
PDQ Cancer Information Summaries
PDQ Clinical Trials Database
Directories of Health Professionals and Organizations, Cancer Care
PDQ Treatment summaries contain prognostic and treatment information on major types of cancer. Health professional versions of the summaries provide detailed information on prognosis, staging, and treatment for each disease, refer to key citations in the literature, and link to abstracts forthe citations. Most PDQ treatment summaries are also available in patient versions, written in easy-to-understand, non-technical language.
Go to the site: http://cancernet.nci.nih.gov/pdq/pdq_treatment.shtml
Look up: Sarcoma, Soft Tissue, Adult
There will be two choices underneath... patient, and health professional. The difference is in the level of writing. Nothing on this site is classified or restricted or under copyright. Download both. Read the patient file first. If you understand it, then go on to read the health professional's file.
These PDQ files are NOT specifically Leiomyosarcoma files. And leiomyosarcomas do not necessarily act or react the same as other sarcomas. But for introductory and general outline purposes, these are useful files. This is a file that you can take to your GP for further discussion and explanation, and to your sarcoma oncologist, too, if necessary. They are updated regularly.
You may request a full list of NCI publications available by email, by sending an email to firstname.lastname@example.org and writing the word "help" in the body of the message.
Using CancerMail, you may request, in English or Spanish:- PDQ Cancer Information Summaries- NCI Fact Sheets- CANCERLIT Citations and Abstracts on selected topics- Recent NCI Press Releases- Selected NCI Publications
|Dealing with Doctors|
From: TALKING WITH YOUR DOCTOR by Suzanne Leider President, The Sarcoma Alliance
Ms. Leider has given us permission to use this article.
Choosing A Doctor
After a diagnosis of sarcoma, choosing the right physician can be a difficult and confusing process. It is important to find a doctor who has experience treating the particular type and stage of cancer you have. This is especially challenging with sarcomas because specialists are difficult to find and often are not located close to home. Visit our list of helpful contacts and resources to assist you in finding a sarcoma specialist that's right for you. The Cancer Information Service (1-800-4-Cancer) can also assist you with treatment facilities including cancer centers and other programs in your area that are supported by the National Cancer Institute (NCI).Several approaches can be taken when a sarcoma specialist is far from home. If you have available financial and social support and are physically strong enough, travelling to one of the sarcoma treatment centers may be the right choice for you. If temporarily moving to a treatment center is not an option, it may be worth your time and money to make a consultation appointment at a sarcoma center. In this way you can obtain treatment advice from a sarcoma specialist and bring this home to your local oncologist for implementation. Sometimes consulting oncologists will write up treatment protocols that your local oncologist can use to guide your treatment. If travelling is not an option at all, have your local oncologist consult by phone with a sarcoma specialist for treatment advice and protocol. In addition to finding a physician who has experience treating sarcoma, it is important that you feel comfortable and confident with your physician. Treating sarcoma can often be a long and arduous experience. Finding a physician who is professionally competent as well as an empathetic and effective communicator is essential. Being treated for cancer is a vulnerable and often terrifying experience and being heard is a key aspect of effective treatment. A good oncologist should be open to questions, interested in concerns, a good listener, and not in a rush.
MEMBERS OF THE HEALTHCARE TEAM
TALKING WITH YOUR DOCTOR
As an integral member of the healthcare team it is important that you take an active role in your care. The following is a list of helpful suggestions for communicating with your physician that will empower you as an informed consumer.
SEEKING SECOND OPINIONS
Seeking a second opinion is not taboo or something to be embarrassed about. In fact, seeking a second opinion is quite common among healthcare consumers and most oncologists welcome colleagues' opinions. By seeking a second opinion, a diagnosis will often be confirmed and you may gain greater understanding of your diagnosis and treatment options. Because sarcomas are very rare and difficult to diagnose, seeking a second opinion from a sarcoma specialist is invaluable. Many oncologists routinely send pathology slides to another pathologist to confirm the diagnosis, especially with rare cancers. Ask your oncologist if this has been done. Fine differences in a diagnosis can have major implications for prognosis and treatment options. In some instances third opinions may be necessary. In addition to seeking a second opinion for diagnosis and treatment information, a second opinion may be warranted if you are having difficulty creating a positive working relationship with your oncologist. Personality conflicts do occur between patients and physicians and staying in a difficult relationship may only hinder care. Like all humans, physicians have varying levels of communication skills and one oncologist may be much more therapeutic than another. Remember that you are not stuck and you do not have to stay in a relationship that is not right for you. Having cancer is tough enough; having a difficult relationship with your physician is an additional strain that you do not need.
|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|