What’s New in Leiomyosarcoma?

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Leiomyosarcoma expert Martee Hensley

Medical oncologist Martee Hensley, an expert in leiomyosarcoma and other uterine sarcomas.

Uterine sarcomas are quite rare, accounting for less than 4% of uterine cancers, which grow in the uterus. About 1,200 women in the United States each year are diagnosed with a type of uterine sarcoma, which includes leiomyosarcoma.

Memorial Sloan Kettering medical oncologist Martee Hensley specializes in the diagnosis and treatment of uterine sarcomas and other gynecologic cancers. We spoke with her recently about the importance of a proper diagnosis and about how treatments are improving the lives of women living with this disease.

How do leiomyosarcomas and other uterine sarcomas differ from other types of uterine cancer?

Uterine sarcomas arise in the muscle or connective tissues of the uterus, whereas the more common type of uterine cancer, called endometrial carcinoma, starts in the lining of the uterus. These two types of cancer have different systems of staging and different treatment approaches. Some high-grade uterine sarcomas can be much harder to treat than endometrial carcinomas and have lower survival rates, even when the disease is caught early.

There are several different types of uterine sarcomas, which vary in their behavior, prognosis, and treatment. The most common type is leiomyosarcoma. Other types include carcinosarcoma, adenosarcoma, low-grade endometrial stromal sarcoma, and high-grade endometrial stromal sarcoma. In addition to being very different from endometrial carcinoma, these sarcoma types are very different from each other. Each has its own method of treatment. For example, some of these tumors may be hormone-sensitive whereas others are not.

Because these tumors can be difficult to diagnose correctly, and because their management strategies can be very complicated, it’s important that women who have them are treated at a center like MSK, which has extensive experience in diagnosing and treating them.

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Could you explain more about why proper diagnosis is so important?

It’s vital to have pathologists who are experts in sarcoma review the pathology slides. At MSK we are fortunate to have those specialists. I work with them closely to understand the specific type of uterine sarcoma each of my patients has.

Our pathologists are experienced in recognizing the fine distinctions of every tumor. This gives us a better understanding of the potential behavior of each patient’s cancer, helps us estimate the risk that the tumor may come back after surgery, and helps us determine the optimal treatment strategy. 

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What are the symptoms of uterine sarcoma?

For all uterine cancers, not just sarcoma, symptoms may include abnormal bleeding or unusual discharge from the vagina between menstrual periods or after menopause. Other symptoms can include pain, an unusual feeling of fullness in the pelvic area, and frequent urination. 

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Are there any risk factors for leiomyosarcoma and other uterine sarcomas?

Uterine sarcomas are very rare diseases. Most women who develop these cancers do not have any identifiable risk factors for sarcoma. Women who are survivors of childhood cancers and who were treated with radiation are at increased risk for developing sarcomas as adults, including uterine sarcomas. But those women are a small minority of cases.

Most women who get this disease develop it in their early to mid-50s, but there is a broad range of ages at which they are diagnosed.

While leiomyosarcomas develop commonly in the muscle wall of the uterus, they can also develop in both men and women in other organs or tissues that have smooth muscle cells.

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How is uterine sarcoma treated?

The most common treatment is surgery, especially when the disease is diagnosed at an early stage and has not spread beyond the pelvis. At a minimum, women need a hysterectomy — removal of the uterus — but may require more extensive surgery depending on how far the cancer has spread.

Uterine sarcoma can metastasize to other parts of the body, most commonly to the lungs. For women who develop metastatic disease, we consider a number of different approaches. If a patient has an isolated metastasis, like a single tumor in the lung or the liver, we can often remove it with surgery or treat it with an interventional radiology approach, such as ablation. The decision about the best approach is very nuanced. For that reason it’s important to consult with a doctor who has experience treating metastatic sarcomas.

Uterine Sarcoma Experts
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For patients whose disease is more widespread, there are a number of different effective chemotherapy regimens that are good at controlling the disease. When one stops working, we usually have other options that we can try.  MSK sarcoma physicians are leaders in the development of new treatment approaches for uterine sarcoma and other sarcomas.

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What are some of the challenges of treating leiomyosarcoma and uterine sarcomas?

It may be difficult to know whether a uterine mass is a sarcoma or not. There are no diagnostic imaging techniques that can reliably distinguish between a uterine sarcoma and a benign uterine growth such as a leiomyoma [fibroid] or a low-grade pathology such as STUMP [smooth muscle tumor of uncertain malignant potential]. For this reason, it’s a good idea for women with abnormal uterine findings to consult with a gynecologic oncologist for proper planning of their surgery.

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Why did you choose to work on uterine sarcomas at Memorial Sloan Kettering?

I feel privileged to care for women who are facing these rare and complex cancers.  It is highly satisfying to help patients understand their disease each step of the way, and to help them make the best treatment choices. Doing this work at MSK means that I am fortunate to work with a deeply experienced team of sarcoma-dedicated specialists representing all aspects of care — pathologists, surgeons, radiation oncologists, medical oncologists, radiologists, and nurses. Our collective skills and experience in the diagnosis and treatment of these rare tumors give us the ability to choose the best treatment for each patient that fits the behavior of that patient’s specific sarcoma.

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Comments

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My mom was just diagnosed with LMS of the Bartholin gland, high grade (3/3) with MRI showing 6.8 cm cystic mass presenting OUTSIDE of the uterus, with leftward displacement of the mid-vagina and the urethra by the lesion. There is a thick enhancing wall inseparable from the right aspect of the vagina, right internal obturator, right aspect of the puborectalis and lower external sphincter and the right hamstring tendon. No worrisome osseous lesions demonstrated. Mild presacral edema is likely reactive. As it is LMS, but not uterine LMS, is Dr Martee Hensley still the right specialist, or is there someone else at MSKCC (or another sarcoma center)? As it was just diagnosed in the past 2 wks and does not appear to have metastasized further yet, we are hoping to find the right specialist as soon as possible to begin intervention.

Dear Tami, we’re sorry to hear about your mother’s diagnosis. MSK has several experts who have experience treating LMS. The referral specialists in our Patient Access Service will be able to direct her to the right specialist. The number to call to reach them is 800-525-2225. You can go to https://www.mskcc.org/experience/become-patient/appointment for more information on making an appointment. Thank you for your comment and best wishes to both of you.

Dear Doctors at MSKCC, I am writing for my baby sister, who at 37 (single and never been with anyone, and may wish to have a child someday) has just been diagnosed with a large 20 cm uterine mass entirely within myometrium (that seems to have grown in less than 2 years), and recommended to have a hysterectomy. We wondered if you would see her for a second opinion, and let us know if somehow her uterus can be safely preserved, without needing to have to go through a higher risk surgery (myomectomy), comparatively speaking. Please can you refer us to someone at your facility asap - we can fly in there from Houston. Thank you most gratefully, Priya

Dear Priya, we’re very sorry to hear about your sister’s diagnosis. If she would like to come to MSK for a second opinion she can make an appointment online or call 800-525-2225. Thank you for your comment and best wishes to you and your family.

Hi. I have recently been told that I have a large 8 cm fibroid in my uterine muscle wall. The ultrasound tech recommended an MRI to exclude cancer as the mass was not present 8 years ago on a previous ultrasound. The MRI tech seems to think it is just a fibroid but cannot definitively exclude LMS due to the rapid growth.

This is what the report said: Enlarged, bulky uterus containing an 8.2 cm left intramural uterine fibroid displacing the endometrium. The fibroid is mildly hypo-vascular, possibly secondary to degeneration. The fibroid is new from a 2011 pelvic ultrasound. Given the fairly rapid interval growth, an underlying uterine leiomyosarcoma is not definitively excluded, although hypovascularity would make this less likely. Gynecology referral is recommended for further evaluation. and management.

I am petrified as now I am being told my only option is to have a hysterectomy. My gyno doesn't think it's anything serious.
Do you think this is cancer? Should I get a second opinion? How reliable are MRI's to diagnose LMS?

Thanks

Dear Natasha, we’re sorry to hear about what you’re going through. If you are interested in coming to MSK for a second opinion, you can make an appointment online or call 800-525-2225. Thank you for your comment and best wishes to you.

My wife passed away from Uterine LMS in 2009. Have there been any advances in this devastating disease in the past 10 years outside of surgery and the standard chemo protocol of Doxil, etc? Just curious.

Dear Robert, we are very sorry for your loss. There are some newer therapies that have been developed, including targeted therapies, and they are successful for a subset of patients, depending on what kind of mutations their tumors have. Thank you for your comment and best wishes to you.