Uterine sarcomas represent a type of soft tissue sarcoma that originates in the uterus (womb). The most common forms develop in the myometrium, the muscular layer of the uterus, while others arise in the endometrium, the lining of the uterus.
Unlike more common types of uterine and endometrial cancers, uterine sarcomas tend to grow and spread quickly. They also have a higher likelihood of recurring after treatment. When they recur, they may affect nearby pelvic lymph nodes and organs, such as the vagina, bladder, rectum, ovaries, and fallopian tubes, or metastasize to distant places like the liver or lungs.
Uterine sarcomas account for only 2 to 5 percent of all uterine cancers, impacting approximately two individuals per 100,000. They most frequently occur in women aged 50 to 70, with black women experiencing a higher diagnosis rate than white women.
Due to their rarity and complexity, uterine sarcomas require immediate care from a specialized team experienced in treating these tumors. At Penn Medicine’s Abramson Cancer Center, leading experts from the Sarcoma Program and the Gynecologic Oncology team collaborate to create personalized treatment plans for uterine sarcoma patients.
Uterine Sarcoma Symptoms
Individuals with uterine sarcomas may experience various symptoms that can differ significantly from one person to another. Many of these symptoms may resemble non-cancerous conditions; thus, you should seek medical attention if you notice:
- A feeling of fullness in the abdomen
- Irregular menstrual cycles
- Pelvic pressure or pain
- Frequent urination
- Vaginal bleeding not related to menstruation or occurring after menopause
Types of Uterine Sarcoma Cancer
Uterine sarcoma includes several distinct types:
- Uterine Leiomyosarcoma: This is the most common type, forming in the myometrium. It grows and spreads rapidly, with black women developing this form twice as often as white women, typically diagnosed at around age 50.
- Endometrial Stromal Sarcoma: These tumors form in the endometrium within supportive stroma tissue. They can be low-grade, growing slowly and often relying on hormones, or high-grade, which tend to be more aggressive.
- Undifferentiated Sarcoma: These rarer tumors can develop in the myometrium or endometrium and are known for rapid growth and spread.
- Adenosarcoma: This uncommon type generally grows slowly and accounts for about 5 percent of uterine sarcomas.
Here is a medical illustration of uterine sarcoma, highlighting the tumor within the uterus.
What Causes Uterine Sarcoma?
Doctors often cannot pinpoint the exact causes of uterine sarcoma, but certain risk factors may increase the likelihood of developing it:
- Previous radiation therapy to the pelvis for other medical conditions.
- Use of hormone therapy, such as tamoxifen, for breast cancer.
Additionally, some individuals inherit genetic changes that raise their risk:
- Hereditary Retinoblastoma: This condition not only increases the risk for retinoblastoma but also heightens the chances of developing certain soft tissue sarcomas, including uterine sarcoma.
- Li–Fraumeni Syndrome (LFS): Although LFS primarily relates to rhabdomyosarcoma, it also raises the risk for other soft tissue cancers, such as uterine sarcoma.
Uterine Sarcoma Diagnosis and Treatment
To diagnose uterine sarcoma, doctors thoroughly examine and discuss your symptoms. They perform a pelvic exam of the reproductive organs and may also carry out a rectal exam.
If you experience unusual bleeding, your doctor may take a tissue sample from the uterine lining (endometrium). They often do this through a dilation and curettage (D&C) or minimally invasive biopsy. However, physicians may need to recommend a hysterectomy to diagnose uterine leiomyosarcoma if it has not involved the endometrium. After the hysterectomy, specialized pathologists analyze the tumor to confirm the presence of cancer. If the tissue sampling or hysterectomy confirms uterine sarcoma, your doctor will recommend imaging tests to check for signs of cancer spread and to stage the disease.
Doctors typically begin treatment for uterine sarcoma by surgically removing the tumor. This procedure usually includes performing a hysterectomy, which may involve the removal of the cervix and potentially the ovaries, depending on the specific type of sarcoma and individual factors. After surgery, your treatment plan may involve monitoring for recurrence or additional therapies such as radiation, chemotherapy, or hormone-blocking medications. Researchers continually explore new treatment options through clinical trials, including targeted therapy aimed at cancer growth pathways and immunotherapy, which utilizes the body’s immune system to combat the disease.
Resource: PennMedicine
