<b>ABBREVIATIONS</b> 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.

<b>PRIMARY</b> site: where the disease started with the wild division of the first mutated cell.

If the <b>PRIMARY</b> tumor sends cells out by the blood or the lymphatic system, and those cells seed and grow, then there will be <b>SECONDARY tumors</b>, or <b>METASTASIS</b>.

If the <b>PRIMARY</b> tumor is removed by surgery, with wide clear margins, it can still reoccur locally. This is called <b>LOCAL RECURRENCE</b>.

Surgical <b>EXCISION</b> or <b>RESECTION</b> is removal of tumor by surgery.

<b>INOPERABLE</b> means that surgery would probably not be able to remove the tumor mass with clear margins.

<b>EN BLOC</b>  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.

<b>CLEAR MARGINS</b> 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.

<b>TRUNCAL</b> = trunk of body, essentially base of neck to groin.

<b>TUMOR WAX OR PARAFFIN BLOCK</b>  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.

<b>TESTING YOUR TUMOR</b> 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.
	
<b>SHELLING OUT</b> 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.

<b>BIOPSY</b> 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. 

<b>DE-BULKING</b>
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 <b>PROGNOSIS</b> is a forecast as to the probable outcome of the disease. In LMS it depends upon tumor <b>GRADE</b>, tumor <b>STAGE</b>, mitotic index, and various patient-determined properties.

<b>MITOTIC INDEX</b> 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.

<b>GRADING</b> 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.

<b>WELL DIFFERENTIATED</b> cancer cells look very much like normal cells.

<b>POORLY DIFFERENTIATED</b> cancer cells look more disorganized and primitive than normal cells.

<b>DE-DIFFERENTIATION</b> 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.

<b>GENOME PROTECTOR</b> 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.

<b>APOPTOSIS</b> 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.  

<b>p53 damage as a cause of cancer</b> 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.

<b>NECROSIS</b> 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.

<b>HEMORRHAGE</b> 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.

<b>MITOSES</b> 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 <b>STAGE</b> 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.

<b>STAGING</b>  Deciding the patient's stage of the disease is <b>STAGING</b>.  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.

<b>REMISSION</b> 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.

<b>5 CENTIMETERS</b> is about the same as 2 inches.

<b>LYMPH NODES</b> are small bean-shaped organs that are found throughout the body; they produce and store infection-fighting cells.

An <b>Oncologist</b> 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. 

<b>Sarcoma oncologist</b> 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.

<b>Sarcoma pathologists</b> 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.

<b>Surgeons</b> 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.

<b>Radiologists</b> -- 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. 

<b>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.</b>