What’s New in Leiomyosarcoma?

By Julie Grisham,

Thursday, July 14, 2016

Leiomyosarcoma expert Martee Hensley
Summary

Memorial Sloan Kettering has a team of experts who specialize in the diagnosis and treatment of uterine sarcomas. Here, medical oncologist Martee Hensley tells us the latest developments in leiomyosarcoma and how our team approach benefits people with these rare cancers.

Highlights
  • Uterine sarcomas are rare, making up only 4% of all uterine cancers.
  • There are many types of uterine sarcoma, and they vary in their prognosis and treatment.
  • MSK pathologists are experts at diagnosis.
  • Many treatments are available, depending on the type and stage of disease.

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
From diagnosis to surgery and other forms of treatment, women with uterine sarcoma are best cared for by a team of specialists familiar with this rare condition. Find out why.
Learn more

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

This is an excellent overview from a renowned specialist. But I'm curious about the inclusion of carcinosarcoma. I thought that was no longer considered a "true" sarcoma.

Suzie, thank you for your comment. We sent your question to Dr. Hensley, who responded, "Carcinosarcomas are cancers that nearly always arise in the gynecologic organ (Mullerian) tract. They have a biphasic appearance with part of the tumor looking like endometrial carcinoma and part looking like sarcoma. The sarcoma portion can look like a number of different types of sarcoma, including sarcomas that would not be expected in the uterus, such as chondrosarcoma. Currently, we consider that carcinosarcomas differ enough from endometrial carcinoma in terms of disease behavior and prognosis that we strive to study them separately. They also differ from leiomyosarcoma (LMS) and thus are not generally included in LMS studies. Like gynecologic carcinomas and gynecologic sarcomas, carcinosarcomas are rare tumors that are best managed by physicians who are clinical experts in gynecologic malignancies."

As a daughter, of a mother who had leiomyosarcoma, I am interested to know if any link has been found to leiomyosarcoma and heredity. I know it worried my mom that she might pass this down to her daughters/granddaughters. Thank you

What treatments do you recommend for leiomyosarcoma in the liver. Treatments so far, each for at least 3 cycles: Gemcitabine, Gemcitabine + Gemcitabine, DOXOrubicin, Trabectdin, Votrient. Tumors have increased in size and number.
Thank you.

Yehuda, thank you for reaching out. We are unable to make specific medical recommendations, but there is currently one clinical trial for leiomyosarcoma at Memorial Sloan Kettering:

https://www.mskcc.org/cancer-care/clinical-trials/15-133

If you would like to make an appointment with a Memorial Sloan Kettering physician, please call our Physician Referral Service at
800-525-2225 or go to https://www.mskcc.org/experience/become-patient/appointment

Thanks for your comment.

So little time so much to do. How does one get accepted into a clinical trial? Because time is of the essence and it is flying...I am curious what criteria is needed for acceptance. Will the first consultation with the physician and all clinical reports available provide an answer? Thank you for all you do.

Dear Christine, every clinical trial has a particular protocol and its own unique set of eligibility requirements. If you are interested in browsing through our open clinical trials, please visit https://www.mskcc.org/cancer-care/clinical-trials where you can search for specific studies using keywords or by disease of interest. You can also learn more about how to decide if a clinical trials is right for you and other helpful information there as well.

If you are interested in a joining a particular study, have questions about any of our trials, or would like to make an appointment with one of our specialists to discuss your treatment options, please call our Physician Referral Service at 800-525-2225. Thank you for reaching out to us.

I HAVE leoiomyosarcoma tumor in my stomach that metastise to my liver and maybe in bones of the pelvis I am looking for treatment .There is a checkpoint study in Seattle opens two weeks .Or should I come to your MSK

Should all uterine sarcoma patients request estrogen and progesterone receptor testing on their tumors? And if positive, which aromatase inhibitors should be tried?

My sister in law was diagnosed in January 2016 with LMS, she is also an insulin dependent diabetic. She underwent Chemotherapy and went into remission. She was clear in PET scan in September and CT scan in December revealed a tumor again and small tumors on the fatty tissue around her intestines and her belly filling with fluid. They have basically told her to look at her quality of life rather than quantity. It is sad to think that this is it. Basically go home and die. I just wish we had found Memorial Sloan sooner, maybe we could have consulted before now, when we feel helpless and with no hope.

Dear Veronica, we are sorry to hear about your sister-in-law's diagnoses.

Coping with advanced cancer is difficult for both patients and caregivers. You and your sister-in-law may find some of this information in this article helpful:
Living and Coping with Advanced Cancer
https://www.mskcc.org/blog/living-and-coping-advanced

Our online community for patients and caregivers is also a good resource to connect with and get support from others who are having similar experiences. To learn more and sign up, please visit https://www.mskcc.org/experience/patient-support/counseling/groups/conn…

Thank you for sharing your thoughts and experience on our blog.

Hi i am preeti from India. I am fighting with metastatic leiomyosarcoma. I have taken chemo MIA, halaven with DTIC,Votrient and now on Yondelis.
Each treatment showed good response for 3 to 4 cycles but after that disease progressed. I am tensed now what next after Yondelis.
Please reply.

Dear Preeti, we are sorry to hear about your diagnosis. If you would like to make arrangements to have one of our specialists conduct a medical records review for you by mail, or make an in-person appointment to consult with one of our physicians about possible next steps in your care, please contact our International Center at international@mskcc.org. To learn more about the services we offer for patients who live outside of the United States, please visit https://www.mskcc.org/experience/become-patient/international-patients. Thank you for reaching out to us.

Hello,
To clarify: if one has had abdominal and transvaginal ultrasounds that show multiple leiomyomas (intramural, submucosal, and subserosal) and thus the endometrium could not be reliably measured, then there is no way to tell if there is some kind of cancer present? Should I see a general gyn next, or go directly to gyn oncology? Not sure it matters, but I am a former patient at MSK and had a total thyroidectomy for papillary thyca, tall cell variant, in 2010. I've been doing well until just recently when I noticed very slight spotting 10 yrs post menopause. I just don't want to waste an oncologist's time if I should have a gyn do initial tests. Thank you!

Dear Laura, we're sorry to hear you're going through this. We sent your question to one of our gynecologic surgeons, and he recommended that you make an appointment with your general gynecologist first. Thank you for your comment, and best wishes to you.

Thank you for taking the time to forward my question to a MSKCC surgeon. It is so helpful to get an expert's opinion on who to see next! I will call the general gynecologist first thing in the morning. Many thanks!
~Laura

Laura, thanks for your comment.

I am now cancer free fpr almost 8 years. I was diagnoside with LMS in 11/2010. I had surgery to remove my uterus cervix and ovaries. I had a lesion in my Gluteal Meduis muslce which was removed after chemo- Gmzar/Taxiter and Doxy. I have nodules in my lungs stable for all ths time. I had a low grade that they even had a hard time verifying was LMS. I still have yearly cat scans I have mine tomorrow. I always get nervous But I feel so lucky. Is this normal? Thanks and GOD BLESS all the cancer patients and wonderful doctors, scientist etc... for all you do!!!

Dear Lorri, we are so glad to hear you're doing well. Thank you for your comment, and best wishes to you.

My mom was diagnose with leiomyosarcoma stage IIB in the pelvis. It was actually a tumor that developed on a residual ovary - she had a hysterectomy and removal of tumor along with some of the abdominal tissue. How long should she wait before treatment? She is also still having a lot of nausea, stomach pain and pain in her abdomen and back at 4 1/2 weeks post surgery - is this normal?

Dear Lisa, we're very sorry to hear your mom is going through this. We recommend that you discuss your concerns and questions with her healthcare team. If she would like to come to MSK for treatment or a second opinion, you can call 800-525-2225 or 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 you and your family.

hello - My mother has LMS and I'm curious to know if anything new has come up recently that we should be exploring.

Thanks

Dear Jason, we're very sorry to hear about your mother's diagnosis. MSK has a number of trials for different types of sarcoma. If your mother would like to make an appointment to learn more, she can call 800-525-2225 or 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 you.

I was diagnosed with uterine leiomyosarcoma at age 39. I underwent surgery and chemotherapy at the time. Today I am 67 years old with no recurrences since then. Is there a research study that could use my background and treatment regimens to help discover more about this disease? I would like to add something to the medical data and research as a result of my experience if it would be helpful. Thank you.

Dear Maggie, we're so glad to hear you're doing well. Unfortunately at the time you had your surgery, tumor specimens were not preserved in a way that would make it easy to obtain any genetic information from them today, even if they were still available -- which they may not be. But we thank you for sharing your story! Best wishes to you.

In 2005 my mother received doxorrubicin plus ifosfamide. I would like to know if it's possible to her receive again doxorrubicin plus OLORATUMAB? Is there any clinical trial for LMS?

Dear Eduardo, olaratumab is now FDA approved for treating sarcoma, in combination with doxorubicin, so patients don't need to be part of a trial to get it. If your mother didn't already receive her maximum dose of doxorubicin, she may be able to get this combination. If she can no longer get doxorubicin, she may be able to participate in a trial using olaratumab in combination with other drugs. Your mother may quality for other trials as well. If she would like to learn more, the number to call to schedule a consultation 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 you and your family.

3 weeks ago yesterday I had an LAVH related to years of dealing with heavy periods, severe cramping and a development of a fibroid. My biopsy results revealed I have Uterine Leiomyosarcoma- stage 1B.
The pathology shows it was positive for estrogen and progesterone receptors. My CT scan shows no metastatic disease at this time. I was wondering if its possible to have a phone consultation with a specialist. My oncologist has given me a few options at this point and I am confused about what is best practice. I am 39 y.o. and still have my ovaries. My options range from simple observation (scan every 3 months) to oophorectomy and/or chemo. My oncologist says it really is up to what I want to do and if the benefits out weigh the risk?
How do you recommend your patients make such decisions?

Dear Stephanie, we're sorry to hear about your diagnosis. We offer medical records reviews to patients who go through our International Center. If you are based in the United States, you would need to meet with one of our doctors in person to get a second opinion from us. If you would like to arrange one, you can call 800-525-2225 or 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 you.

When a uterine mass is detected in an imaging study, many ordinary gynecologists commonly presume that the suspect mass is a benign growth such as a "fibroid" tumor. Most of the time they are correct, but occasionally they are wrong. What should a woman do if she is diagnosed with a uterine growth from an imaging study and her gynecologist recommends surgical treatment based on the presumed diagnosis that the mass is almost definitely benign? Is it time to seek a second opinion from a cancer specialist before proceeding with any surgical treatment? What could be the consequences of proceeding with the recommended surgical treatment from a non-cancer specialist, and then discovering after surgery that the presumed benign diagnosis was completely wrong? Sadly, this scenario happens much too often -- especially with uterine sarcomas. What can be done to make it happen less often?

Dear Bill, in this type of situation, it may be best to consult with a surgical oncologist. Experts at National Cancer Institute-designated cancer centers would be a good place to start. You can find a list at https://www.cancer.gov/research/nci-role/cancer-centers. Thank you for your comment.

Today was told I have three leiomyosarcoma Tunis in uterus. I'm 63. I also have nodules in my abdominal sac. Doc wants to do surgery and try to get everything out.. I am unable to eat cause tumor pressing on intestines.. love to send CT and audio to get second opinion... plus I would love to get in touch with some of people on this. Hope to hear

Dear Janis, we're sorry to hear about your diagnosis. If you would like to come to MSK in New York for a second opinion, you can call 800-525-2225 or go to https://www.mskcc.org/experience/become-patient/appointment for more information on making an appointment.

If you are interested in getting in touch with other patients who have been through this diagnosis, you may want to join Connections, our online support group for cancer patients and their families. You can learn more at https://www.mskcc.org/experience/patient-support/counseling/groups/conn…

Thank you for your comment, and best wishes to you.

Hola hace tre semana me diagnoticaron con leiomisarcoma uterino cual seria la mejor quimioterapia para este tipo de cancer.

Querida Dulce, sentimos mucho el diagnóstico que te han dado.

El hospital Memorial Sloan Kettering tiene un equipo de expertos multidiciplinarios disponible para ofrecer varios tratamientos para cáncer del útero, incluso sirugía, radiación, quemoterapia, terapia hormonal, y terapias investigacionales. Visite esta página para más información sobre este tipo de cáncer y sus tratamientos: https://www.mskcc.org/cancer-care/types/uterine-sarcoma/treatment.

No podemos recomendar un tratamiento especifico a traves de nuestro blog. Sería mejor consultar con uno de nuestros especialistas. Para hacer una cita con uno de nuestros médicos, llame a nuestro Servicio de Referencia Médico al 800-225-2225.

Gracias por su comentario.

my 35 years old wife felt a swelling on her right abdomen area. upon CT we were told its a dismoid tumer and must be removed. we did the surgery and the doctor says that the tumor is completely removed. the initial pathology report says its spindle cell tumor. the second report says its benign. upon discussions the pathologist says could lead to LMS. I am confused. can I send the report to your organization for a second opinion.

thanks

Hello. My wife was diagnosed with uterine leiomyosarcoma in March 2015 following a full hysterectomy. For a while she was all clear and we hoped it may be a STUMP but it returned in her kidney, which was removed in March this year. She also has a very small 7mm tumour in her lung but there is also now a metastasis in her spine for which she insisted on radiotherapy, as the specialist hospital we attend were not going to offer any treatment? To date this is the only 'treatment', other than surgery, my wife has received or been offered. My wife is a nurse and therefore very pro active in trying to look ahead and look after herself and spot any ongoing health issues. And she reads all your information and advice. I believe we may need a 2nd opinion on her current state of health and whether treatment should be offered asap. I would really appreciate your thoughts on this and also any hope you can offer regards treatments available or on-going research. Thank you for your time and all you do in researching uterine leiomyosarcoma

Dear Stevie, we're very sorry to hear about your wife's diagnosis and recurrence. If your wife would like to come to MSK for a second opinion, you can call 800-525-2225 or go to https://www.mskcc.org/experience/become-patient/appointment for more information on making an appointment.

Based on your email address, it looks like you may not be in the US. If that is the case, you can contact our International Center about arranging for a review of her medical records. You can email international@mskcc.org or go to https://www.mskcc.org/experience/become-patient/international-patients/… for more information. Thank you for your comment, and best wishes to both of you.

I have been diagnosed with Leiomyosarcoma. and have gone through Doxorubicin and Olaratumab (4 cycles), and the tumor has not changed much Your website says that surgery was the best option, so I met with one of your surgeons. I did not take well to the chemo. The surgeon feels I need more chemo. I am just wondering why the surgery cannot be done to remove the tumor, and the chemo to get rid of any other remaining cancer. Just wondering!!!

Dear RP, this is something you should discuss with your MSK healthcare team. They should be able to answer any questions you have. Thank you for your comment, and best wishes to you.

Hi. I had a series of imaging test in Feb - Mar 2018 that revealed 2 bowel intussusuceptions and a 10mm x 11mm mass on liver. Pathology report stated mass was hemangioma. Determination due to my age, 65, no need for removal of same. Was waiting for insurance approval on small bowel study. Emergency surgery mid May to examine small bowel. To surprise of all 5cm x 5cm tumor plus suspicious growth with necrosis that may have been lymph node. Surgeon very skilled, removed with wide margins, 12 lymph nodes, and did not open to examine. Did thorough visual examine of entire small bowel. Diagnosis LMS, that would typically present in uterus. Went to sarcoma center, mass on liver is now 49mm x 40mm. Radiologist report stated metastasis, needle biopsy of liver required for confirmation. For biopsy, is it essential to do at sarcoma center? Suggested chemotherapy of Doxorubicin and Dacarbazine. If needle biopsy determines LMS in liver and therefore metastasis, plan suggested chemo first, surgery later. Do I need to physically come MSK for a second opinion? I am considering a trial, but I have only found Level I trials. Do you know of an appropriate TRIAL II or TRIAl III? Thank you in advance, for your consideration of my request for information and insight.

Dear Kathleen, we're sorry to hear about your diagnosis. If you would like to get a second opinion from an expert at MSK, you would need to come to New York to meet with one of our doctors in person. The exception would be if you live outside the United States. In that case, you could arrange for a records review through our International Center.

You can go here for more information on making an appointment: https://www.mskcc.org/experience/become-patient/appointment

You can go here to learn about a records review through the International Center: https://www.mskcc.org/experience/become-patient/international-patients/…

If you are in the US but not able to travel to New York, you may want to consult with an expert at a National Cancer Institute-designated cancer center that is closer to where you live. You can find a list here: https://www.cancer.gov/research/nci-role/cancer-centers

Thank you for your comment, and best wishes to you.

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