The following article was written in 2006. The views expressed do not necessarily represent those of the Sarcoma Alliance.
By Suzie Siegel
Volunteers and physicians are working hard to improve the treatment of women whose sarcomas arise in their reproductive tract. For the first time, the Sarcoma Alliance sent a representative to the Society of Gynecologic Oncologists’ annual conference, which drew more than 1,700 people to Palm Springs, Calif., March 22 – 26, 2006 (www.sgo.org). Board member Dave Murphy and I talked to more than a hundred doctors, none of whom knew about sarcoma support organizations, but they welcomed brochures, fliers and wristbands and were happy to discuss sarcoma at length.
One of them was Dr. Michael Friedlander, a gynecologic oncologist from Sydney, Australia, who is president of the International Gynecologic Cancer Society. Its annual meeting will be Oct. 14-18 in Santa Monica, Calif. (www.kenes.com/igcs-11). He welcomed sarcoma advocates to attend and promised that sarcomas would be discussed.
Although carcinomas dominated discussions at the SGO conference, some presentations included sarcomas. Of more than 600 posters, approximately 20 mentioned sarcoma. However, gynecologic oncologists would like to do more studies on sarcoma, but because it’s rare, they have a hard time finding patients and funding, said Dr. Edward Trimble, head of the National Cancer Institute’s Gynecologic Cancer Therapeutics & Quality of Cancer Care Therapeutics. The conference was his last as a board member of the Gynecologic Cancer Foundation (GCF), which is associated with the SGO (www.thegcf.org).
Dr. Bobbie Gostout, a member of the GCF executive committee and chair of GCF’s communications committee, foresees important studies coming to fruition.
“There’s a buzz on the street that this will be a big year for sarcoma”, she said.
Most studies of gynecologic sarcomas are done through the Gynecologic Oncology Group, one of nine cooperatives sponsored by the National Cancer Institute to do clinical trials, Trimble said. This institutional structure makes it harder to collaborate on research with doctors in sarcoma programs, said Gostout, a gynecologic oncologist from the Mayo Clinic in Rochester, Minn., who was interviewed by telephone. But she said: “The door is not closed to that possibility – not at all. Other groups can adopt our protocols. Cooperation between groups is common. … Most of us are very comfortable working with sarcoma colleagues [and] sharing expertise.’
Doctors and researchers who are not gynecologic oncologists also are welcome to submit abstracts on gynecologic sarcomas for consideration at SGO conferences, said GCF Executive Director Karen Carlson in a phone interview from the foundation’s offices in Chicago. Additionally, the GCF welcomes grants of $25,000 or more from individuals, organizations and companies to research a particular subject, she said. No one has donated for sarcoma yet. “It’s a wonderful way to pay tribute to someone. [The donor] would get all the credit, and we’d do all the work. If the GCF had the money, it also would love to print a brochure on gynecologic sarcomas, Carlson said.
The foundation will publish its fourth “State of the State of Gynecologic Cancers’ report in September, said Marsha Wilson, GCF’s director of communications. The 2005 report offered little good news for sarcoma patients, with no mention of studies on AP23573, sorafenib, anti-angiogenesis and aromatase inhibitors. The report isn’t limited to the work of gynecologic oncologists, but it only includes studies on gynecologic cancers, Gostout said. Many sarcoma studies include patients whose cancer arose in different parts of their bodies.
Web sites for “women’s cancers” often discuss cervical, ovarian and vaginal “cancers’ when they mean carcinomas. “Sometimes I shudder and say, This is not the correct terminology,“ said Gostout, who has published on uterine leiomyosarcoma (ULMS). (Uterine cancers can be either carcinomas or sarcomas. Carcinomas arise from the epithelial cells and sarcoma from the connective tissue.)
The GCF created the Women’s Cancer Network (http://www.wcn.org/) in 1997. It mentions sarcoma under the heading of vaginal and vulvar cancers, but not under ovarian and cervical cancers. Gostout said that the WCN is written for a fifth- or sixth-grade level so that it can reach more people, and that most people don’t know the terms “carcinoma’ and “sarcoma.’ She said she didn’t realize that the wording was confusing to some sarcoma patients, and that the site might add a line or two, noting that women also can get sarcoma in other anatomical areas.
The WCN added an entire section on uterine sarcomas a few months ago. The site has added information on the use of MRI’s and ultrasounds to distinguish benign fibroids from leiomyosarcomas.
Some information needs to be updated and expanded. For example, the site mentions only two chemotherapy drugs for LMS: Adriamycin and Temodar. Other drugs, such as Ifex, Gemzar and Taxotere, are used more commonly than Temodar.
LMS is described as “cancer within the muscular wall of the uterus.” ULMS is often found inside “fibroids,” as the GCF’s 2005 report notes. Saying it is within the muscular wall may confuse women who get a “fibroid” removed, only to have the pathology report find LMS.
In places, the wording is the same for both the uterine sarcoma and endometrial carcinoma sections. For example, follow-up care is the same for endometrial carcinomas, and the FAQ’s relate primarily to endometrial carcinomas. The WCN has three pages of links to articles on endometrial carcinomas. Recently, it has added a general article on cancer to the uterine sarcoma section. The WCN lists links to other cancer organizations, but none to sarcoma groups.
Wilson said the WCN will fix anything inaccurate, and that the uterine sarcoma section will be updated and expanded, as are other sections. “Funding drives our ability to add information,’ Gostout said. “You do what you can with the resources you have.” For at least $25,000, an organization or company can attach its logo to the site, she said, and the money is used to add information. For example, a pharmaceutical company just donated $40,000 to update information on colorectal cancer.
For more information on women with gynecologic sarcomas, please go to the June 2006 issue of ESUN:
Some Web sites that discuss “women’s cancers” make little or no mention of sarcomas or sarcoma doctors. But their advocates have been willing to listen.
The Web site for Our Bodies, Ourselves (http://www.ourbodiesourselves.org/) added information at my suggestion. Sheryl Silver said she would consider adding information to her Web site for Johanna’s Law: The Gynecologic Cancer Education and Awareness Act (http://www.johannaslaw.org/).
At the SGO conference, advocates for “women’s cancers” warmly greeted their sarcoma counterparts. Evelyn Lazare, executive director of Ovarian Cancer Canada (www.ovariancancercanada.ca), talked to us at length about strategy. Newsletter editor Katie Allen said she would add my story to Eyes on the Prize (www.EyesOnThePrize.org), which already includes other sarcoma patients. The site has posted new information on sarcoma, such as the difference between ULMS and endometrial stromal sarcoma.
Mary Jackson Scroggins, president of In My Sister’s Care, asked for more information, as did Linda Koteen, director of the ovarian program for SHARE (http://www.sharecancersupport.org/).
The nonprofit National Comprehensive Cancer Network (NCCN) and the government-run National Cancer Institute (NCI), whose Web sites provide information for professionals, separate uterine sarcomas from all other soft-tissue sarcomas. Sarcoma advocates have asked why gynecologic oncologists write the NCCN guidelines for uterine sarcomas, while doctors in sarcoma programs writes guidelines for other soft-tissue sarcomas.
“Why is it that we are ‘kept in the dark’ about how different a sarcoma cancer is than other gynecologic cancers?’ asked Joan Connor, a registered nurse for more than 30 years who had ULMS. “And, why oh why, isn’t our particular sarcoma treated as a sarcoma by those who put forth national guidelines for treatments of cancer?”
She later reported to others with LMS that Dr. John Brooks, chair of pathology for Pennsylvania Hospital in Philadelphia, said the NCCN’s soft-tissue sarcoma panel will write guidelines for uterine sarcomas, probably starting next year. The Sarcoma Alliance for Research through Collaboration (SARC) will help translate the professional guidelines into material for patients. Brooks’ announcement came at the Leiomyosarcoma Hugfest in April in Cherry Hill, N.J.
This year’s guidelines can be found at:
This raises the question: Why are uterine sarcomas treated differently? For example, the imaging guidelines for ULMS are almost the opposite as those for retroperitoneal LMS. If a low-grade retroperitoneal tumor has been removed, the soft-tissue guidelines recommend a physical exam, plus a CT of the abdomen and pelvis, every three to six months for two to three years. “Consider chest imaging” was added this year. Lung metastases usually don’t cause symptoms until they have grown significantly. Nevertheless, the NCCN guidelines are less stringent on chest imaging even though they note “soft-tissue sarcomas most commonly metastasize to the lungs.” The NCCN guidelines for uterine sarcomas recommend imaging of the chest, but assume pelvic exams are sufficient for the abdomen and pelvis.
For patients, it may be confusing that different specialists research sarcomas, and they have separate organizations, Web sites, conferences, reports and recommendations. At times, it seems as if doctors are dueling over patients, said Connor, the nurse who had ULMS.
The GCF report urges women to see gynecologic oncologists, and never mentions that sarcoma programs exist. The WCN site allows patients to search for gynecologic oncologists as well as “related” doctors. Although I live only a few minutes from the H. Lee Moffitt Cancer Center in Tampa, when I searched for a related doctor, the site turned up no one in Moffitt’s sarcoma program.
Gostout reiterated that gynecologic oncologists work well with sarcoma colleagues, and that GCF is open to collaboration. Doctors at the SGO conference said they had no problem referring a patient to a sarcoma program.
When I contacted the NCI’s Cancer Information Service, two information specialists said I needed to see a gynecologic oncologist, not a doctor in a sarcoma program. Carolyn Waddel, a ULMS patient in Fort Myers, Fla., wrote the U.S. Surgeon General about this issue. Deborah Pearson, chief of public inquiries for the CIS, wrote back “that the NCI does not usually set guidelines or make specific recommendations, including advocating a particular type of cancer specialist for the treatment of any specific cancer.’
Pearson noted that the NCI’s Sarcoma Progress Review Group issued a report in 2004 that “advocates the use of multidisciplinary sarcoma centers for the treatment of all sarcomas” ( http://planning.cancer.gov/library/2004sarcoma.pdf ).
The report mentions a raft of oncologists, but none in gynecology. A prominent sarcoma doctor who asked to remain anonymous said gynecologic oncologists often treat sarcomas as if they were carcinomas. “Most gyn oncs don’t really know how to treat sarcomas.
“I used to think a lot about how we can get sarcomas to be seen by specialists, but I think that it’s hopeless in our system, where there is no incentive to pass patients along to experts. Most patients don’t have a chance in our current system …”
In the past, some women – including myself in 2004 – have had trouble getting to see sarcoma doctors at comprehensive cancer centers. But centers seem more cooperative lately.
Some, such as Moffitt and M.D. Anderson Cancer Center in Houston, emphasize that patients can ask for an appointment in their sarcoma programs without a doctor’s referral. Doctors in different programs cooperate on patient care. They also stress to patients that, after consultation, they can return to doctors at home for treatment.
Dr. Barbara Goff, a gynecologic oncologist in the Seattle Cancer Care Alliance (SCCA) and SGO secretary-treasurer, said she has been working for about two years with Dr. James Butrynski, a medical oncologist in the sarcoma program.
“We have been teaming up to apply the multidisciplinary approach to sarcoma,” she said. “I make James come in and do the pelvic exam with me.”
“Similarly, our research programs are integrated whenever possible and information exchange is frequent between programs,” said Dr. Marc Stewart, the SCCA’s medical director.
Stewart prefers that women with sarcomas of the reproductive tract see gynecologic oncologists first. “Sometimes patients are transferred to the care of a medical oncology sarcoma specialist for management of metastatic disease. … When chemotherapy becomes complex or the patient prefers to see a medical oncologist with sarcoma expertise, we make sure a transfer of care to the proper specialist occurs.”
At MSKCC, patients may see a gynecologic oncologist, a medical oncologist specializing in gynecologic cancers, and/or a medical oncologist in the sarcoma program.
“I work very closely with the medical oncologists who treat non-uterine soft-tissue sarcomas,” said Dr. Martee Hensley, a medical oncologist who specializes in uterine sarcomas in the gynecologic oncology service. She was interviewed through email. “We have a highly collaborative program that provides women with multidisciplinary expertise.”
In their PRG report, sarcoma doctors conclude: “Everyone should be treated by someone with expertise in sarcoma. If patients can’t be treated, at least their doctors could consult with sarcoma experts.”
Who is a sarcoma expert? There is no official certification for a “sarcoma specialist.” Trimble pointed out that surgical, radiation and medical oncologists and pathologists can all be sarcoma experts. Gynecologic oncologists are surgeons. Those who see many sarcoma cases can be considered sarcoma doctors, too, he said.
Gynecologic sarcomas are rare enough that it would be hard for a gynecologic oncologist to see only sarcoma patients, he said. On the other hand, gynecologic oncologists tend to see the same sarcomas: LMS, carcinosarcoma, and endometrial stromal sarcoma.
Instead of counting how many sarcoma patients a doctor sees, prospective patients may want to look at how many of those women have survived, said Dr. Niki Nikrui, a gynecologic oncologist in Weston, Mass.
She worries that sarcoma programs at large cancer centers emphasize clinical studies, without enough attention to what would benefit individual patients.
In response to criticism of gynecologic oncologists not consulting with sarcoma programs, Nikrui said: “Communication and cooperation is a two-way street. … I don’t think any treatment can be single-handed. It has to be teamwork.”
Trimble said it is “perfectly appropriate” for a woman to see both a gynecologic oncologist and a medical oncologist in a sarcoma program. Nor does he have a problem with a patient seeking a second opinion.
“But they don’t need a sixth opinion,” he said, explaining that some women delay treatment as they go from doctor to doctor, trying to figure out what to do.
Because women with gynecologic sarcomas generally see a gynecologist or gynecologic oncologist first, he said, it makes sense for a gynecologic oncologist to do their initial surgery and manage their follow-up care. A medical oncologist might be better for patients whose sarcomas have spread or recurred, he said.
No matter how sarcoma expertise is defined, the sarcoma PRG report states that many patients, if not most, receive unacceptable care.
“Diagnosis is delayed in many patients by the lack of experience of primary physicians, who often attribute the initial mass to common benign lesions,” the report says.
Patients may understand only that they have cancer; they may not know what sarcomas are or that sarcomas often are treated differently from carcinomas. They may not know that sarcoma doctors exist, or they may trust their current doctor to treat them. They may lack the desire, time or money to travel to a large cancer center with a sarcoma program, the report says.
“Additionally, physicians and surgeons in small- and moderate-sized hospitals often want to maintain control of patients … for economic and other reasons,” the report says. “However, many physicians do not appreciate how complex the care of sarcoma patients has become and are not aware of the many alternative care strategies available.”
A patient may get tests that are not recommended, such as fine-needle biopsies. She may not get proper follow-up tests. Her tumor may grow large and deep, becoming inoperable and/or metastasizing. Her surgeon may not know to take wide margins. She may receive chemotherapy unlikely to kill sarcoma cells.
These mistakes affect how long a woman with sarcoma will live, and the quality of her life. Her anger at misdiagnosis and incorrect treatment may hurt her mental health.
Doctors have a hard time getting enough patients for studies to determine the best ways to diagnose and treat sarcomas, the report says.
“Ideally, since uterine leiomyosarcoma is a rare disease, all women with this disease would be managed in the setting of a clinical trial,” Hensley said. “Information from such studies is critical to helping women with this disease live longer and better.”
A framework for cooperation in research and treatment could serve as a model for other rare diseases, the PRG report says. But the NCI cannot afford to implement the PRG recommendations, Trimble said. “It’s hard to do new things when you don’t have new money.”
In December 2005, President Bush signed a bill that provided $31 million less for cancer research than the year before, according to the American Association for Cancer Research. “This was the first hard cut since the passage of the National Cancer Act in 1971. More recently, the president announced his proposed budget for fiscal year 2007 – it slashes an additional $40 million from the already reduced 2006 funding level.”
With permission from the owners of the Association of Cancer Online Resources list for LMS (www.acor.org), I solicited comments about gynecologic oncologists. Most respondents asked for anonymity.
Some women wrote about how well gynecologists and gynecologic oncologists have treated them, and how their doctors sent them to a sarcoma program or helped them get a second opinion.
One man said his wife sees a gynecologic oncologist who has published LMS research. The doctor sent his patient to a sarcoma program for a second opinion, and in general, seeks consultations whenever he wants more expertise.
“He is compassionate, caring and explains things to us,” the husband wrote. Gynecologic oncologists “may not all be knowledgeable or even competent in treating LMS, but we shouldn’t throw them all out with the bathwater.”
Rosalie Peipert, who lives in Spotswood, N.J., has survived LMS and endometrial stromal sarcoma for almost 25 years. She has great respect for her gynecologic oncologist, but recommends patients get a second opinion at a sarcoma center.
“Most gyns are in hick towns and treat the patients awful.” She said doctors often dismiss patients after surgery, without follow-up care. If the cancer comes back, the women “don’t even know it until they have symptoms.”
Meg Thorburn, a professor of epidemiology in Ontario, Canada, is a three-year survivor of ULMS. She disagrees with those who think gynecologic oncologists are fine for sarcoma surgery, without input from sarcoma doctors. The highly respected head of the gynecologic oncology division at a medical school operated on her.
“The gyn onc did no imaging prior to my surgery, and had refused to do any
routine surveillance on me other than manual pelvic exams (the one he did three days before my surgery failed to detect the 4 cm tumor in my vaginal sidewall) and sporadic chest X-rays.
“So, my first recurrent tumors were a surprise found during a scheduled oopherectomy. [That was] my second ‘whoops’ procedure – the first being, of course, my hysterectomy.”
During surgery for her recurrence, “he didn’t even bother to resect my sigmoid colon, where one of the tumors occurred. [He] just kind of skimmed it off. So, of course, I had inadequate margins, and the tumor recurred in less than eight months.
“The sarcoma oncologist to whom I transferred my care after that fiasco
made sure, with this subsequent recurrence, that I was operated on by one of
the best abdominal oncologic surgeons in Ontario, who knew that sarcoma requires wide margins and brought in other (urology and vascular) surgeons as necessary. Seems simple, eh? I’m afraid knowing my story has just fueled my sarcoma oncologist’s previous distrust of gyn oncs.”
One woman sees a gynecologic oncologist who thinks that, since her ULMS was found early, she is cured and does not need any follow-up CT scans. “They are merely to patronize me and keep me at bay.”
She insisted on scans after seeing a doctor in the sarcoma program at Moffitt who recommended them.
One woman sees a chief of gynecologic oncology at a major hospital. After nine months, he told her that she didn’t need scans for another year. Her radiology oncologist insisted on scans at least every six months.
“I believe others with ULMS have the same problem, possibly because of the unrealistic [NCCN] guidelines that we’re working to update,” she said.
One woman who ended up at MSKCC said her original gynecologic oncologist removed her pelvic lymph nodes, which wasn’t necessary. Although he had trained at M.D. Anderson, she said, she thinks he hadn’t kept up to date on sarcomas.
Another woman said her gynecologic oncologist gave her no hope. “I was sobbing and asked him when I should commit suicide. My husband was about to reach over the man’s desk to choke him.” She asked for a referral to MSKCC, and the doctor said, “Why bother?” She now sees a caring and knowledgeable doctor in MSKCC’s sarcoma program.
“I ‘interviewed’ five or six gyn oncs at respected Philadelphia hospitals,” she said. “Not one knew more than the name ULMS.”
A registered nurse who has worked in the recovery room for 20 years said her gynecologist did tests, but dismissed her continual reports of pain and other symptoms, as well as the advice of a radiologist, for months. She finally had surgery, which revealed ULMS.
Her doctor asked her to find an oncologist in their hospital, even though MSKCC was an hour away. She thinks loyalty and ego influence referrals. “He knew it would be the fight of my life, and he wanted me to be treated with this rare cancer at my small hospital.”
She now sees a sarcoma oncologist at MSKCC. Her sarcoma had recurred in her pelvis and spread to her lungs and liver. After treatment, she is now free of cancer.
“I have read many other stories like mine, stories of horror and inconsolable fear. It should not happen if we could only get more awareness out regarding these rare sarcomas, and get to the beginning of the cycle … at the desk of the M.D. that treats us. He needs to listen, inform himself, update his knowledge, and never play the odds. He needs to fight for the tests needed to diagnose, instead of being afraid of his bottom line. He needs to humble himself, and send patients to the experts in the field of sarcomas.
“Too often I have heard surgeons refer to the surgeons at large specialty hospitals as the ‘hot shots.’ I am sure they would run to a ‘hot shot’ if they or their family member had the need.
“I only hope that my journey through hell will help someone dealing with this cancer – because someone has listened to this painful account of a patient that has to spend the rest of her life knowing that she had a chance to be cured if only someone had listened.”
Joan Connor, who lives in Port Republic, N.J., and works in an operating room, saw a gynecologist for symptoms. He told her the tumor on her MRI scan was benign. “He told me to go home and stop thinking about my pelvis.“
She went to another gynecologist, who ended up calling a gynecologic oncologist. She loves them both, she said. Once she was diagnosed, however, she wishes they had referred her to a sarcoma center before beginning treatment. She has since consulted with a sarcoma doctor at MSKCC.
“The GP who used to ‘set’ a fractured leg now sends his patient to an orthopedic specialist. The broken hand gets sent to the ‘hand surgeon.’ The guy with a cardiac arrhythmia goes to see a cardiologist. Even the local gynecologist calls in a gyn oncologist for the treatment of a gyn cancer. The sarcoma specialty should be looked at as just another step up in the ‘specialty chain.’
“All sarcoma patients, no matter what the primary site, should be automatically sent to a sarcoma center.”
Suzie Siegel was a newspaper reporter and editor for 18 years before being diagnosed with vaginal LMS. She likes and respects her sarcoma and gynecologic oncologists.