Rhabdomyosarcoma

All information given here is only of a general nature, tumor therapy always belongs in the hands of an experienced oncologist!

Synonyms

Muscle tumor, soft tissue tumor, soft tissue sarcoma

English: rhabdomyosarcoma

definition

A rhabdomyosarcoma is a rare soft tissue sarcoma, the origin of which originates in the striated muscles (rhabdo = transverse striation; myo- = muscle). Rhabdomyosarcoma is a (sub) form of sarcoma, which is categorized according to bone, soft tissue or connective tissue cancer.

Summary

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood. There are three different forms of rhabdomyosarcoma, which differ in the shape of their cells when examined under a microscope. They should be listed below and briefly described in their cell form:

• Embryonic rhabdomyosarcoma: immature, spindle-shaped cells
• alveolar rhabdomyosarcoma: multinuclear giant cells (several cell nuclei); In addition, the rhabdomyoblasts show transverse cytoplasmic striations.
• polymorphic rhabdomyosarcoma: rounded, elongated cell nuclei; pronounced nuclear polymorphism.

Rhabdomyosarcomas are principally found in the area of ​​striated muscles, but most often they are found in the head and neck area, the urogenital tract and the extremities.

Rhabdomyosarcomas make themselves felt differently in terms of symptoms. The form of the symptoms depends on the location of the sarcoma. Pain and possible functional impairments are among the most common symptoms of such a disease.

If your child has such symptoms, the doctor will usually arrange for an X-ray examination, which, if suspected, can usually lead to further examinations. For example, a tissue sample (Biopsy) serve. As mentioned above, a distinction is made between different forms of rhabdomyosarcoma, so that if the disease is present, attention must also be paid to the cell shape for more precise determination (microscopic examination).

The therapy is always individually tailored. It ranges from radical surgery to adjuvant chemotherapy or radiotherapy (radiation therapy). Which form of therapy should be sought individually always depends on the individual clinical picture.

The prognoses are also very individual and depend in a special way on the localization, the extent and the shape (see above) of the sarcoma. The five-year survival rate averages 60%. It can be described as rather unfavorable, since rhabdomyosarcomas recur (renewed tumor growth) within one to two years. The therapy carried out also has a great influence on the respective prognosis.

Staging

As soon as a child is suspected of having rhabdomyosarcoma, further examinations are arranged. As soon as the disease has been confirmed it must be found out whether the tumor cells have already expanded into other parts of the body, as this is the only way to plan the therapy individually and adequately. These examinations serve the so-called staging.

There are different systems for dividing the stages. They are usually based on size, initial location and the extent of (possible) expansion.
The stages will be discussed in more detail below. The stages do not correspond to so-called progressive stages, but rather serve to describe the individually pronounced disease state.

Stage 1:
Location of the cancer:

• • in the eye

  • near the sex organ

  • in the urinary bladder

  • in the area of ​​the head and / or neck

Stage 2:

• • The rhabdomyosarcoma was found in one part of the body. The body parts, however, are not those listed in stage 1.

  • The diameter of the tumor <5 cm.

  • No expansion into the lymph nodes.

Stage 3:

• • The rhabdomyosarcoma was found in one part of the body. The body parts, however, are not those listed in stage 1.

  • The diameter of the tumor ≥ 5 cm.

  • The cancer spreads to the lymph nodes that are near the rhabdomyosarcoma.

Stage 4:

• • Metastasis of the rhabdomyosarcoma at the time of diagnosis.

Relapse:

• • Relapse - formation after therapy for rhabdomyosarcoma. Relapses can recur in the same place or affect other parts of the body.

localization

Rhabdomyosarcomas are particularly common in the head / neck region, the urogenital tract (urinary tract) and the extremities. In principle, rhabdomyosarcomas can be located anywhere on the body.

Metastases form in particular in the lungs and bones, in the brain and in the pelvic organs.

Symptoms

Rhabdomyosarcomas make themselves felt differently in terms of symptoms. The form of the symptoms depends on the location and extent of the sarcoma.

Urogenital (lower urinary tract) cause rhabdomyosarcoma very often stomach pain, Haematuria (= blood in the urine), dysuria (= Painful urination), Constipation (= constipation) and vaginal bleeding in female patients, but testicular swelling in male patients.

Cause in the extremities Rhabdomyosarcomas on the one hand painful, but also painless swellings. Functional restrictions can also be part of the symptoms of rhabdomyosarcomas.

As already mentioned above, the localization is a decisive factor for the type and severity of the symptoms. In stage 1 rhabdomyosarcomas, for example, the eyeball protrudes or shifts, including the Nasal breathing can be hindered or the jaw swell or hearing disorders appear.

diagnosis

If a rhabdomyosarcoma is suspected, for example due to the symptoms, imaging procedures are usually used for diagnostic determination. This includes, for example, X-ray examinations, but also magnetic resonance imaging (MRT) or scintigraphy. A biopsy (= fine tissue examination) can be considered for diagnostic confirmation and for more detailed planning of therapeutic measures.

forecast

The prognostic chances of a long-term cure depend on the stage. The rule is: the higher the stage, the worse the prognosis. While in Stage I the chances of recovery at about 80% can be seen, they lie in the Stage IV on average only at 20 percent.

The prognoses are therefore dependent in a special way on the location, the size and the possible formation of metastases.

The average five-year survival rate is around 60%.