
<b>"How long have I got, doc?" </b>
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.

<b>THIS INFORMATION MUST NOT BE TAKEN AS PREDICTIVE IN ANY WAY. </b>

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.

Roger
November 2002