Leiomyosarcoma

The leiomyosarcoma (Greek Li'o, Leio, , smooth, gentle ' and μυς, mys, muscle ', σάρκωμα, sarcoma to σάρξ, sarx "flesh ", " soft tissue " and om " tumor " ) is a malignant ( malignant ) tumor, which emanates from the smooth muscle. He makes, among other things about 1 % of all malignant Gebärmuttergeschwülste. This tumor usually occurs after age 30 and has its peak age in the sixth decade of life. The symptoms are usually nonspecific, it comes with localization in the uterus to a rapid uterine enlargement with bleeding. Leiomyosarcomas may also be of smooth muscle at any other point, eg, also come from the Haarbalgmuskeln and thus occur in principle in the total body surface.

Pathology

In the embryonic development of the so-called mesoderm developed including the locomotor apparatus (bones, muscles), the soft tissues and the blood and lymphatic system. These tissue types are designated in the mature state as mesenchymal tissue. Almost all malignant tumors arising from these mesenchymal tissues are known as sarcomas. The leiomyosarcoma metastasized predominantly via the blood.

While it was previously thought that the leiomyosarcoma may degenerate from a benign leiomyoma, recent studies show that this is unlikely. Likewise there is no evidence that previous irradiation is a risk factor.

Rarely, leiomyosarcomas under a hereditary retinoblastoma on, also found in a few tumors changes in the RB tumor suppressor gene.

Diagnosis

Due to the non-specific symptoms, the diagnosis is usually an incidental finding, if for other reasons, an operation was necessary.

The leiomyosarcoma is usually indistinguishable from leiomyoma in the surgical specimen, possibly occurring necrosis may provide an indication of the dignity. Furthermore, infiltration of malignancy in the surrounding tissue, such as for localization in the uterus into the myometrium, endometrium or parametrium.

In histology, there are multiform cell nuclei and giant cells. The most relevant criterion for the diagnosis of leiomyosarcoma remains the Mitosereichtum (more than 10 mitoses in 10 fields of view at 400 × magnification ) and simultaneously occurring polymorphism of the cells ( cellular polymorphism ).

Therapy

Since the leiomyosarcoma insufficient responsive to chemotherapy and radiation therapy, the operation is at the center of the therapy. There are currently no mandatory standards since the leiomyosarcoma one hand belongs to the heterogeneous group of sarcomas and on the other hand its occurrence is very rare.

In about 30 % of cases, metastases are found in the ovaries with localization in the uterus, so the Splenic of the ovaries is attached ( ovariohysterectomy ). In 3-5 % of diagnosed leiomyosarcomas, there are also metastases in the large network. In 4-35 % of patients, there are lymph nodes in the pelvis and adjacent to the main artery. However, removal of these lymph nodes seems to bring no prolongation of life with it. The leiomyosarcoma results in 50 to 70% of cases to a recurrence of the malignant tumor, usually within the first two years. It metastasizes, particularly in the lungs, and has a poor prognosis.

Only the low-grade leiomyosarcomas, which belong to the less dangerous types, have a better survival rate. They walk away slowly, so that tumors often recur after 10 years. The affected patients have a long life expectancy. Overall, it is based on a 5 - year survival rate of 50%.

Swell

• Riede / Shepherd: pathology. ISBN 3-1368-3303-1
• Robbins ' Pathology ' ISBN 978-1-4160-2973-1