Uterine Leiomyosarcoma (ULMS)
Uterine leiomyosarcoma is a malignant tumor found in the smooth muscle lining the walls of the uterus. It can also refer to leiomyosarcoma tumors in the vagina, ovaries and other gynecological areas. According to the FDA and other expert sources, uLMS can be found in as many as one in 350 women with uterine complaints related to tumors widely known as fibroids.
Symptoms of uLMS vary from woman to woman and can include:
Heavy periods
A rapidly growing fibroid
Abnormal vaginal bleeding
Abnormal or mucousy vaginal discharge
Pelvic pain or pressure
Pain or discomfort during sexual intercourse.
Abdominal pain or pressure
Abdominal bloating and distention
Change in bladder or bowel habits
Blockage of urinary flow
Uterine leiomyosarcoma is often mistaken for uterine fibroids. In fact, in most cases, uLMS is assumed to be uterine fibroids. Fibroids, also known as uterine leiomyomas, are benign smooth muscle tumors of the uterus that affect many women. Mainly asymptomatic, fibroids are a benign condition that may not require treatment. However, fibroids can grow in size and cause the symptoms listed above. Any woman diagnosed with fibroids must tell her gynecologist to treat each fibroid as a possible uterine leiomyosarcoma tumor.
DIAGNOSIS
Uterine leiomyosarcoma is typically a surprise discovery after a routine surgery to remove fibroids or the uterus. Preoperative diagnosis of uLMS is extremely difficult because a biopsy can miss the tumor cells and MRI scans can not tell the difference between fibroids and uLMS tumors. In 2014, the FDA warned against the routine use of morcellation of the uterus, due to the risk of the fibroid actually being leiomyosarcoma. Complete surgical removal of the tumor is the definitive treatment for uterine leiomyosarcoma. En bloc removal of the uterus should be performed in all cases of fibroids. In most cases of uterine leiomyosarcoma diagnosis, removal of the ovaries and fallopian tubes (a bilateral salpingo-oopherectomy) may be recommended. Therefore, it is essential for all women with fibroids to have an informed discussion with their gynecologists and NOT rule out the possibility of uLMS.
OCCURRENCE RATE OF UTERINE LEIOMYOSARCOMA
Uterine leiomyosarcoma occurs in approximately 1 in 10,000 women in the general population. When the symptoms listed above are present, uLMS is found in nearly one in 350 women with an even higher incidence in women above the age of 40.
CAUSES
Researchers know that uterine leiomyosarcoma is the result of cell DNA mutations. However, what causes that mutation is not absolutely known. These genetic changes could spontaneously develop, or be environmentally induced by radiation treatments at a younger age, or exposures to carcinogenic agents such as pesticides and weed killers. A breast cancer drug called tamoxifen has been linked to uterine leiomyosarcoma. Other researchers suspect this gene mutation could be inherited from a parent. There have been documented cases of mother/daughter and sister/sister uterine leiomyosarcoma. There are also a few related disorders which run in families: retinoblastoma, Li-Fraumeni syndrome, Gardner’s syndrome and Werner’s syndrome.
SURVIVAL RATES
For patients with stage I and II uLMS (the cancer has not spread beyond the uterus) the 5-year survival rates can be as high as 80%. In women with stage III and IV uLMS (the cancer has spread to other parts of the body) the prognosis is extremely poor. In these women, 5-year survivals approach 10-20%.
LONG TERM SURVIVORS
These numbers need to be updated. With newer treatments and more research, there has been in the last ten years, an increase in long term uterine leiomyosarcoma survivors. You can read their stories in our LMS Community Blog.
TREATMENT
Uterine leiomyosarcoma is an aggressive cancer that is difficult to treat. The only definitive chance of survival comes with adequate and safe surgical removal of the tumor. Adjuvant therapies (therapy given after tumor removal) such as chemotherapy and radiation therapy have been used to combat the disease. However, these treatments have not proven to reduce the chance of recurrence or metastases. Treatment when there is tumor with chemotherapy and radiation is poor, however the results can vary greatly. Uterine leiomyosarcoma is more chemo sensitive than leiomyosarcomas of the retroperitoneum, large blood vessels and limbs. The use of gemzar with taxotere (referred to as gem/tax) is the standard first line chemo for uterine leimyosarcoma, if surgery cannot be done. Uterine leiomyosarcoma cells have been known to remain dormant for even 15-20 years and then start to grow. Because of its aggressive nature, surveillance should be frequent (3-4 times a year) and long term (annually.)
HORMONE RECEPTORS, TESTING AND BLOCKERS
The rate of estrogen receptor (ER) and progesterone receptor (PgR) expression varies among uLMS tumors. However, it has been found to be present in 40%-80% of cases. The test to determine uLMS hormone receptor expression is simple and can be ordered by your doctor. The pathologist stains slides from your original tumor. They estimate what percentage of the tissue took up the stain indicating estrogen and progesterone receptors on the tumor tissue sample. uLMS is considered hormone-receptor positive if the tumor contains substantial amounts of estrogen and/or progesterone receptors. If a tumor is determined to be estrogen-receptor positive, it means that estrogen fuels the cancer cells growth within the tumor.
ULMS ESTROGEN RESEARCH INFORMATION
The goal for treating estrogen and/or progesterone positive uterine leiomyosarcoma tumors is to block the production of estrogen. In pre-menopausal women, this treatment can include removing the ovaries (oophorectomy) or taking medication such as Zoladex or Lupron. If a patient is undergoing chemotherapy, nothing may need to be done as the chemotherapy may already be blocking estrogen production. Aromatase inhibitors (AIs) such as Arimidex, Femara, and Aromasin have been used to block estrogen being made in post-menopausal women.
Side effects of AIs include bone loss, hot flashes and sometimes joint pain. To counter the side-effects of AIs, supplements (vitamin D3, and calcium) and weight bearing exercise are recommended. Additionally, a DEXA scan should be performed to test bone density every two years. All side-effects and treatment options should be discussed with your doctor.
For more information on uterine leiomyosarcoma and estrogen, watch video #7 on uLMS of the LMS Boot Camp webinars.
UTERINE LAPAROSCOPIC MORCELLATION
If your hysterectomy was performed using morcellation, your risk of recurrence is much higher. We advise you to seek legal council immediately. Please watch the LMS Boot Camp video #7 for details on why this is so important. Also, see Campaign Against Morcellation, a Facebook public page about advocacy against morcellation
Dear uLMS Survivor,
Ask you oncologist to have your (stored) primary tumor tissue tested for hormone receptors – estrogen & progesterone (Er & Pr.) If your tumor is highly positive for hormone receptors – then you want to reduce the amount of estrogen in your body, because it is stimulating your tumor cells to thrive.
Give you doctor the research information on uLMS and hormones.
If you still have ovaries which are still producing estrogen – consider the option of having your ovaries removed. Your ovaries are the major producer of estrogen. You should discuss this carefully with your doctors. There are also drugs your doctor can prescribe to stop ovary production of estrogen without surgery.
If you are POST menopausal – surgically, naturally or because of chemotherapy – you can request an aromatase inhibitor (AI.) The AI blocks the postmenopausal estrogen production that is made in your fat cells and glands. Discuss side effects/benefits with your doctor. There are three AIs: Femara (letrozole), Arimidex (anastrazole) or Aromasin (exemestane.)
You do NOT want to take tamoxifen. Several studies have found tamoxifen use to be associated with abnormal growth of uterine tissues and also associated with various uterine cancers, including uLMS.
In my own case, I have been NED (no evidence of disease) since 2004, after surgical removal of my second lung met. I then requested to have my tumor tested for hormone receptors. It expressed 99% positive for both estrogen and progesterone. I convinced my doctor to remove my still functioning ovaries. I then took Arimidex, an aromatase inhibitor (AI) for the following seven years. I have been tumor free since. This is significant due to the aggressiveness of stage IV, uLMS. I have never had any radiation or chemotherapy, just surgeries and the AI.
Since 2006, several of us with uLMS on the LMSDR Facebook group, have documented our success stories using AI’s and shared them with sarcoma researchers. We got their attention. It has resulted in two clinical trials for letrozole and uLMS. Today, the NCCN guidelines recommend AIs for uLMS with positive receptors.
I hope you follow in our footsteps and advocate for much needed research on uLMS. You can do this by sharing this information with new uLMS patients and helping to raise research funding with the Leiomyosarcoma Support & Direct Research Foundation (LMSDR.) A dear friend of mine who passed on from uLMS used to remind me, “If you want something, you have to work for it, not wish for it.
Working not Wishing,
Sharon Anderson MSW
President of Leiomyosarcoma Support & Direct Research Foundation
uLMS survivor since 2002
