Mueller’s mixed tumor is a malignant tumor in women. It occurs predominantly in the uterine area. Disease of the fallopian tubes, ovaries, and mesentery are also described in the literature.
What is Mueller’s mixed tumor?
Mueller mixed tumor refers to malignant mesodermal mixed tumors located in the ovary or ovarian area, respectively. The term Müller mixed tumor is obsolete. Today, these tumors are referred to as carcinosarcoma or simply mixed tumor, respectively. The name carcinosarcoma comes from the fact that the tumors contain both carcinoma components and sarcoma components. Carcinomas are neoplasms that originate in epithelial tissue. Sarcomas are of mesodermal origin. This means that they originate from the cells of the so-called mesenchymal supporting tissue.
Causes
Müller’s mixed tumors represent 25 to 60 percent of all sarcomas of the uterus. However, barely three percent of all malignancies of the female reproductive organs are sarcomas. Overall, the disease is very rare. By 2005, just 50 cases of the tumor had been described. Most women who develop a Mueller’s mixed tumor are older than 65 years. The tumors develop from the cells of the Müller duct. This is located in the uterine mucosa. However, it is unclear why the cells of Müller’s duct degenerate. What is certain is that in Müller’s mixed tumor, the blood and lymph vessels are rapidly affected. Thus, metastasis already occurs in very early stages. Already in stages I and II, lymph node metastases are found in this tumor in up to 35 percent of cases.
Symptoms, complaints, and signs
Often, the tumor remains asymptomatic for a long time. When symptoms do occur, they resemble those of benign fibroids. Affected women often notice abnormal bleeding from the uterus. Menstruation can usually be ruled out at the age when Müller’s mixed tumor occurs. In women who are still menstruating, the tumor may be manifested by menstrual pain and increased menstrual bleeding. In addition, there may be pain in the lower abdomen. Rapid enlargement of the uterus is also an indication of a malignant process. Patients may notice that they gain weight within a short period of time despite their usual diet. There may even be visible swelling in the abdominal area. In advanced stages of the disease, abdominal and gastrointestinal symptoms also occur due to displacement of abdominal organs or metastasis to other organs.
Diagnosis and disease progression
If a tumor is suspected in the area of the reproductive organs, the attending physician first performs an ultrasound examination (sonography). If this does not yet provide any information, a computer tomography and magnetic resonance imaging can also be performed. If a tumor is discovered during these examinations, fractional curettage can be used to determine whether or not it is a Müller mixed tumor. In fractional curettage, tissue is removed from the uterus using a sharp curette. To avoid cell mixing between cells of the cervix and uterus, the cervical canal is scraped before curettage. Then, the tissue obtained during curettage is examined histologically.
Complications
Since this disease is a cancer, no general course can usually be given. This depends thereby very strongly on the expression and the spread of the tumor. It is also possible that the life expectancy of the affected person is reduced due to the tumor. Those affected primarily suffer from relatively severe menstrual pain. These also last longer than usual and are also accompanied by relatively heavy bleeding. Many women also suffer from depression or mood swings, which can lead to complications with their partners. Furthermore, those affected gain weight in a short time and also suffer from swelling in the face. If this tumor is not treated, it can spread to the other regions of the body. Discomfort in the lower abdomen or stomach may then also occur. Treatment of this tumor is not associated with complications. It is removed with the help of a surgical procedure.The remaining components can then be removed with the help of radiation or chemotherapy. Side effects may possibly occur during this process.
When should you see a doctor?
Muller’s mixed tumor occurs exclusively in women. Since they belong to the high-risk group and the disease can have a fatal course, they should exercise increased vigilance in case of physical or health changes. The sooner a doctor is consulted, the better the chances of recovery. Women are well advised to attend regular check-ups with a doctor as a matter of principle. Abnormalities are noticed during these examinations, which leads to early detection and thus to rapid treatment of the tumor. Irregularities and inconsistencies of the monthly menstruation of a sexually mature woman are signs of an existing disorder. They need to be investigated and treated if they persist over a long period of time or increase in intensity. If increased bleeding occurs, or if intermittent bleeding occurs, a visit to the doctor is necessary. In case of cramps, pain in the abdomen or disturbances during sexual intercourse, a doctor should be consulted. Swelling in the abdomen, a feeling of tightness or changes in the appearance of the skin should be presented to a doctor to clarify the cause. An unwanted increase in weight without a change in food intake is considered a warning signal from the organism. If there is a general feeling of indisposition, a decrease in physical performance or a feeling of illness, a doctor is needed. In case of digestive disorders or irregularities in going to the toilet, a clarification of the complaints is necessary.
Treatment and therapy
Mueller’s mixed tumor is operated on immediately after diagnosis. During surgery, the abdominal cavity is carefully examined. Then, a so-called lavage cytology is taken. Then the uterus, ovaries and fallopian tubes are completely removed. The large mesh (omentum majus) and the lymph nodes in the abdominal cavity are also removed. Radiation therapy and chemotherapy are not very effective for this type of tumor. Radiation therapy does not have a positive effect on survival, but it may appear to reduce local recurrences. Chemotherapy does not improve survival at all. If the tumor has metastasized or even if there is recurrence, it can only prolong survival. For chemotherapeutic treatment of Müller’s mixed tumor, agents such as carboplatin, doxorubicin, docetaxel, paclitaxel or gemcitabine are used. However, the prognosis of Müller’s mixed tumor is generally considered to be rather poor. It depends on several factors. On the one hand, the sarcoma content of the tumor plays a role. Secondly, the tumor stage and the location of the tumor are relevant. In stage I, the 5-year survival rate is less than 50 percent. In more advanced stages, the rate is only 25 to 30 percent. Prognosis also worsens in cases of deep infiltration of the uterine muscles, pelvic lymph node metastases, and tumor ingrowth into surrounding blood vessels.
Prospect and prognosis
The prognosis for Müller’s tumor is generally poor, but it depends on several factors. The 5-year survival rate depends on the percentage of sarcoma tissue. It is lower the higher this percentage is. Overall, the decisive factor is whether the specialists succeed in removing all the tumor tissue in a surgical procedure. If only a few residual (remaining) tumor cells remain in the body after surgery, the prognosis is quite good. However, the tumor stage also plays a role in the success of the surgical intervention and thus the prognosis. For example, the 5-year survival rate for a stage I Müller mixed tumor is between 40 and 50 percent. As the disease progresses, this drops to 25 to 30 percent. In addition, the extent or depth to which the tumor tissue has infiltrated the myometrium (middle layer of the uterine wall), the surrounding blood vessels and the cervix uteri is important for the prognosis. This is because as the tumor grows into these structures, the prognosis worsens. In addition, metastases (metastases) in the lymph nodes in the pelvic cavity (so-called pelvic lymph nodes) have a negative effect on the prognosis. If left untreated, a Muellerscher mixed tumor leads to death in any case.
Prevention
Since it is not yet known why the cells in the uterus degenerate, the tumor cannot be prevented. Because the chances of survival decrease as the tumor progresses, early detection and rapid removal of the tumor is very important. Early detection examinations can detect precancerous lesions at an early stage. In Germany, annual screening exams are free of charge for women over the age of 20. During the screening examinations, a cell smear is taken from the uterus. This so-called “Pap test” is used to quickly detect cell changes in the uterus. If possible, this should be done before cervical cancer develops. This provision for early cancer detection applies only to healthy women. In the event of symptoms, women should not wait until the next screening appointment, but should see a doctor directly. Such alarm symptoms include spotting and bleeding outside of menstruation or after menopause, pain in the lower abdomen or pain during sexual intercourse. Also, if other diseases of the uterus, such as fibroids, are known, more frequent examinations by the gynecologist should be performed.
Follow-up care
The average time until tumor recurrence is less than two years for Mueller’s mixed tumor. Therefore, in the following three years after therapy, a checkup is recommended every three months. During this check-up, the vagina is examined, taking into account the history of the disease. Likewise, the pelvis is palpated, since one in four recurrent tumors manifests there. If medications have been prescribed, the side effects and interactions, which may be severe, must be discussed with physicians during these clinic visits. For the patient himself, it is recommended to engage in sporting activities. This makes it possible to reduce pain and lighten the mood. Furthermore, moderate sport has a positive effect on the prognosis. This strengthens the performance of the cardiovascular system and the immune system. Sports activities can be started at the end of the therapy and continued afterwards. Furthermore, a diet is recommended, which should be worked out together with a doctor or nutritionist. The change of diet should then be enforced according to the recommendation of the attending physicians. This can alleviate the discomfort of chemotherapy, but also improve the prognosis. Because this aggressive tumor is associated with a poor prognosis, psychological support for those affected is recommended. This can be supported by psychologists or take place in a self-help group.
What you can do yourself
Patients who have been diagnosed with a Mueller’s mixed tumor require prompt medical treatment. Medical therapy can be supported by sports measures, among other things. Regular exercise already helps reduce pain and improve mood during initial therapy. Even after completion of treatment, moderate exercise improves the prognosis by strengthening the performance of the cardiovascular system and immune system. An adapted diet further supports the therapy. Cancer patients should work out a suitable diet with the doctor in charge or a nutritionist and implement it consistently. This can reduce discomfort, especially during chemotherapy, and improve the prognosis overall. Accompanying therapeutic counseling is always indicated. Since Müller’s mixed tumor is a malignant tumor, many patients suffer from anxiety and panic attacks. These must be worked through in cooperation with a psychologist or within the framework of a self-help group. In addition, regular visits to the hospital are necessary, especially in the case of unusual symptoms or side effects and interactions caused by the prescribed medication. Finally, tumor patients should take it easy and avoid physically or mentally strenuous activities, especially in the first weeks of therapy.
