Sarcomas are a group of cancers that originate in the connective tissue of the body and can occur in various parts of the body. Connective tissue includes muscle, fat, and cartilage tissue, as well as bone, blood vessel tissue, and other tissue that supports and connects the body. Fortunately, sarcomas are relatively rare. Accordingly, gynecological sarcomas are also very rare. This, in turn, has the disadvantage that there is little qualified information and hardly any educational material available for those affected. Understandably, this causes uncertainty. Therefore, the treatment of sarcoma disease is always a major challenge for both the patient and the medical team, which can be mastered with a great deal of mutual trust.

Sarcomas of the female genital tract are a subfamily of soft tissue sarcomas. Even though only two percent of malignant gynecological cancers are gynecological sarcomas, they are unfortunately among the most aggressive forms of tumor. They usually develop in the uterus, ovaries, vagina, or vulva. However, not all gynecological sarcomas are the same.

There are various subtypes that differ in terms of their fine tissue composition:

• The most common gynecological sarcoma is leiomyosarcoma, which develops in the smooth muscle walls of the uterus.
• The second most common sarcoma is endometrial stromal sarcoma, which also develops in the uterus.
• Adenosarcomas are characterized by a mixture of different cell types, including glandular cells (adenocarcinoma-like cells) and connective tissue cells (sarcomatous cells).
• Carcinosarcomas (see “Did you know?”)

In principle, gynecological sarcomas tend to affect older women, but younger women can also develop them.

There are risk factors for many malignant forms of cancer, such as familial and/or genetic factors, environmental conditions, and unhealthy behaviors. For example, we know that smoking significantly increases the risk of developing lung cancer. No such classic risk factors have yet been found for sarcomas. Hereditary causes, such as those that can occur in breast or ovarian cancer, are also not usually found in gynecological sarcomas. Only very rare sarcomas have a genetic link to their development.

In recent years, however, certain chemicals have been identified as general risk factors for the development of sarcomas. Scientific studies show an increased risk of developing sarcoma, especially with intensive contact with the chemical “polyvinyl chloride,” known as ‘vinyl’ or “PVC.” One risk factor that has been proven beyond doubt is medical radiation therapy. People who have undergone radiation therapy for another tumor disease, such as breast cancer, have a fivefold increased risk of developing angiosarcoma, a vascular tumor. Nevertheless, this is extremely rare.

As with many other gynecological cancers, gynecological sarcomas are problematic because they do not cause any symptoms for a long time, or cause symptoms that may not initially appear to be gynecological. For example, a sarcoma can cause lung metastases, which then impair lung function and cause shortness of breath. Unfortunately, this means that sarcomas are often diagnosed at a late stage, which is why they often have a poor prognosis.

Possible symptoms of gynecological sarcomas include:

• Abnormal vaginal bleeding: This is one of the most common symptoms. It can be irregular, heavy, or prolonged menstrual bleeding. In postmenopausal women who no longer menstruate, any vaginal bleeding should be considered abnormal.
• Pain in the pelvic area: Women with gynecological sarcomas may experience pain or pressure in the lower abdomen or pelvic area, which may also be noticeable during sexual intercourse, for example.
• Vaginal discharge: Unusual, often watery or bloody discharge from the vagina may occur.
• Weight loss and fatigue: In advanced sarcomas, weight loss and fatigue may occur, which may be due to the spread of the tumor and the body’s energy consumption.
• Enlarged uterus or ovaries: In some cases, the tumor can cause the uterus or ovaries to increase in size and become palpable in the pelvic area.
• Abdominal swelling or enlargement: Some gynecological sarcomas can cause abdominal swelling, which is often associated with a feeling of fullness or pressure.

Many of the symptoms mentioned can also occur in other gynecological diseases and do not necessarily indicate sarcoma, so a thorough examination and diagnosis is crucial. Nevertheless, women who have persistent or unusual symptoms in the pelvic area, especially postmenopausal women, should have them examined. Timely diagnosis and treatment are crucial to increase the chances of recovery.

The diagnosis of gynecological sarcomas requires a thorough examination and various diagnostic procedures. Here are the steps that are typically performed in the diagnosis of gynecological sarcomas:

• Medical history and physical examination: The doctor begins by taking a detailed medical history, asking about symptoms, family history of disease, and other relevant information. A thorough physical examination, including a gynecological examination, is performed.
• Imaging techniques: Various imaging techniques can be used to visualize the tumor and its extent, including:
  • Ultrasound (sonography): Ultrasound can be used to assess tumors in the uterus, ovaries, or other gynecological organs.
  • Computed tomography (CT) or magnetic resonance imaging (MRI): These procedures provide a detailed view of the internal organs and help assess the size and location of the tumor.

• Tissue sampling (biopsy) and histopathological examination: Tissue sampling (biopsy) is necessary for a definitive diagnosis. A sample of the suspicious tissue is taken and examined under a microscope to determine whether it is a sarcoma tumor. The cells and tissue structures are analyzed in detail to determine the type of tumor and the degree of malignancy.
• Staging and further examinations: After the diagnosis, staging is performed to determine the stage of the tumor and whether it has already spread to other organs. This may include additional examinations such as a PET-CT scan or a lymph node biopsy.

Because they are so rare, treating gynecological sarcomas is challenging. It is important for those affected to place themselves in the hands of a team of gynecologists, oncologists, surgeons, pathologists, psychologists, and therapists who are experienced in dealing with this disease. This is the only way to determine the best treatment options for each individual situation. This is because sarcomas are completely different in terms of their tumor biology, and their drug and surgical treatment differs significantly from that of other malignant tumors.

When planning treatment, it is important to develop a comprehensive concept that includes the individual surgical procedures, systematic analysis of the tumor tissue, and possible individual follow-up therapies, such as chemotherapy and radiation therapy or the latest targeted cancer therapies.

Surgery

When the disease is first diagnosed, surgical removal of the sarcoma is the most important therapeutic measure. In this procedure, the surgeon always attempts to remove as much of the sarcoma as possible, including healthy tissue, to ensure that no malignant cells remain in the body. In the early stages, surgery may be the only treatment necessary.

Chemotherapy

If metastases are already present, surgery usually takes a back seat, as the disease has already spread throughout the body. In this case, a treatment strategy that involves the entire body must be used. Chemotherapy, which can take effect throughout the body, is best suited for this purpose.

Unlike other types of cancer, relatively little new research and few new treatment approaches have been developed for sarcomas and gynecological sarcomas.

The following approaches are worth mentioning:

Targeted therapies: These focus on specific cell structures rather than cell division, as is the case with traditional chemotherapeutic agents. They may involve antibodies or so-called “small molecules” that are small enough to penetrate the sarcoma cell and influence cell metabolism there. The side effects differ from those of chemotherapy. A few years ago, a targeted therapy strategy using the substance pazopanib was approved. Administered as a tablet, this tyrosine kinase inhibitor prevents the formation of new tumor vessels. As a result, the tumor tissue is not supplied with enough blood and oxygen, which can lead to the sarcoma cells “drying out” and dying.

Treatment with trabectedin: For several years now, an effective treatment has been available for recurrent leiomyosarcoma—a type of sarcoma in the uterus—using the novel substance trabectedin. This substance is extracted from sea squirts. On the one hand, it acts similarly to conventional chemotherapy on the DNA of tumor cells. On the other hand, it also has a targeted effect. This includes, for example, preventing the formation of new blood vessels that are important for the tumor (neoangiogenesis inhibition).

Hyperthermia means overheating and refers to a specific therapeutic approach in oncology, which is not entirely new. It involves heating tumor tissue to a temperature higher than body temperature. This is intended to kill the tumor cells more effectively, as they already have a higher metabolism than normal body cells, which is further stimulated by the heat, causing the tumor cells to run out of oxygen. In addition, the increased cell metabolism means that the tumor cells absorb the active ingredients of chemotherapy more effectively. Hyperthermia should therefore not be seen as a stand-alone therapy, but rather as a treatment that is most effective in tumor tissue when combined with chemotherapy. However, there is still relatively little experience with this treatment in gynecology, so for the time being it remains a very experimental concept for affected women and is not yet part of standard therapy.