Sarcoma: A Frequently Misdiagnosed and Rare Cancer
Most people haven’t heard of sarcoma. After all, sarcomas are rare, comprising less than 2% of all cancers in adults and accounting for about 16,000 new cases in 2019. Misdiagnoses of sarcomas, however, are extremely common.
Sarcomas can occur anywhere in the body. In most cases, sarcomas develop in soft tissue such as muscle, fat, vessels, and nerves (80%) but can also occur in bone (20%). Symptoms mimic a variety of common benign conditions, complicating early detection. As a result, sarcoma patients frequently report visiting several types of doctors before receiving an accurate diagnosis.
Common red flags of sarcoma cancer include a lump, bump or bruise in any part of the body; a mass that a) measures two inches or more, b) is enlarging, or c) is located deep within the tissue. The tumors are sometimes painful or tender (especially in the case of bone sarcomas) but are just as likely to be painless. Signs of potential sarcomas may warrant consultation with a sarcoma specialist. Encouragingly, if a sarcoma tumor is removed while still localized, the five-year survival rate is high.
Currently, there are no definitive tests to detect sarcoma. If sarcoma is suspected, appropriate imaging techniques are critical before removing the mass. Though no radiology can differentiate between benign and malignant tumors, magnetic reasoning imaging (MRI) has been deemed more reliable than ultrasonography (2), as it provides optimal soft-tissue contrast and better delineates the size and location of a mass.
Among women, sarcomas may develop in the reproductive system and can be mistaken for common conditions such as benign fibroids, perimenopause, or other types of uterine cancers.
Uterine sarcomas account for 4% of all cancers of the uterus (3) and can be found in fibroids, the endometrium, and other parts of the reproductive tract. Additionally, while rare, the occurrence of uterine sarcoma within fibroids may be greater than previously thought; recent research indicates that women undergoing fibroid surgery have a 1:350 chance of uterine sarcoma. (4) “Women have to be mindful that fibroids can harbor a possible sarcoma, and if they have a diagnosis of fibroids they should discuss their individual risk with their physician,” says Dr. Stephanie Terezakis, Department Head of Radiation Oncology at the University of Minnesota.
Uterine sarcoma survivors urge women to visit a doctor if they notice any symptoms or changes near the reproductive region. Concerning symptoms include abnormal vaginal bleeding, pelvic pain or pressure, change in bladder or bowel habits, blockage of urinary flow, a rapidly growing fibroid, abnormal vaginal discharge, and pain or discomfort during sexual intercourse. Suzie Siegel, former board member of Sarcoma Alliance and survivor of vaginal sarcoma, explains, “Sarcoma is so rare and doctors aren’t expecting it. My best advice for women with symptoms such as bleeding in between periods, pain, odor, or other changes is to get it checked out. Even if the symptoms feel embarrassing. Keep after them,” she says.
“Many women ignore these types of things or explain it away as stress or being tired, but I’m a nurse, and I just couldn’t ignore the weird symptoms that kept coming back.”
Over several months, sarcoma survivor and endoscopy nurse, Dondee Murray, experienced heavy vaginal bleeding, low back pain, and a feeling of fullness in the lower abdomen and vagina. She also discovered a small lump under her belly button. “Many women ignore these types of things or explain it away as stress or being tired,” she says, “but I’m a nurse, and I just couldn’t ignore the weird symptoms that kept coming back.” Even after visiting multiple doctors, patients may receive few answers and symptoms can easily be attributed to other, more likely causes. Debbie Miller, endometrial stromal sarcoma cancer survivor, explains, “Once they found the tumor, all my symptoms made sense—it was a classic case. But originally the doctor disregarded it because some of my symptoms are common complaints of women my age.” Dr. Stephanie Terezakis, Department Head of Radiation Oncology at the University of Minnesota encourages patients to be their own advocate: “If a patient feels that they have concerning symptoms they can ask for further testing beyond an exam in the doctor’s office. Imaging studies and biopsy can reveal a potential sarcoma and these tests can be pursued if needed.”
Above all, women should be aware that surgically removing a mass in pieces, as in some minimally invasive laparoscopic procedures involving morcellation, carries a significant risk of disseminating cancerous cells throughout the abdominal cavity. Experts advise masses be removed whole, if possible. And uterine sarcoma survivors counsel women that if any type of morcellation is offered they should carefully research and discuss the risks and alternative procedures.
Misdiagnoses and treatment delays are common for this rare cancer, and greater sarcoma awareness is needed among the medical community and patients to allow for early detection and better patient outcomes. Patients are encouraged to be persistent in pursuing answers if their symptoms continue in order to avoid unnecessary delays in diagnoses. Toni Kitteredge, survivor of myxoid liposarcoma of the abdomen, advises, “You know your body more than anyone else; trust your intuition. Use your common sense. If something doesn't feel right, then it’s probably not. There are so many possible things it could be. Most of the time it will not be sarcoma, but there is still that chance.”
About Nikki littlefield Miller
Nikki Littlefield Miller, M.A., is a freelance writer based in Minneapolis. Trained as a social scientist with over 20 years experience, she uses research and storytelling to interest readers in a wide range of topics. As a volunteer for Rein In Sarcoma (RIS), the Upper Midwest’s largest advocacy group for sarcoma cancer awareness, she has written several articles ranging from survivor stories to features in publications such as MN Medical Magazine.
About Rein in Sarcoma
Rein in Sarcoma (RIS) is a 501(c)(3) nonprofit with a mission of using love, hope, safety and support to serve those impacted by sarcoma cancer. RIS supports sarcoma patients and their families, promotes awareness of sarcoma cancer symptoms and treatments among medical professionals, and funds critical research conducted by leading medical institutions. Founded in 2001, Rein in Sarcoma has raised over $2 million for sarcoma research and founded a sarcoma scholarship program for medical students. For more information see reininsarcoma.org
Sources:
(1) Charoenlap C, Imanishi J, Tanaka T, Slavin J, Ngan SY, Chander S, Choong PF. Outcomes of unplanned sarcoma excision: Impact of residual disease. Cancer Med. 2016; 5(6);980-988. doi:10.1002/cam4.615
(2) “Ultrasonography has been shown to be less reliable than magnetic resonance imaging for further investigation.” Brouns F, Stas M, De Wever I. Delay in diagnosis of soft tissue sarcomas. Eur J Surg Oncol 2003;29: 440-5. [PubMed] [Google Scholar]
(3) https://www.mskcc.org/cancer-care/types/uterine-sarcoma
(4) https://www.fda.gov/medical-devices/surgery-devices/laparoscopic-power-morcellators