Das rhabdomyosarkom im kopf-hals-gebiet: Eine zusammenstellung einiger kasuistiken, therapiemoglichkeiten und prognosen

Background: Soft tissue sarcoma represent approximately 7% of all neoplasms in children up to 14 years. The most common type (approx. 50%) among them is the rhabdomyosarcoma (RMS). Within the head and neck, which accounts for approx. 35% of all RMS, the tumor can be found ubiquitously. Preferred localizations are the orbita, the paranasal sinuses, and the soft tissue of cheek and neck. The prognosis of RMS was formerly poor but has markedly improved since defined multimodal therapy was established in the Seventies (for example the Intergroup Rhabdomyosarcoma Study, IRS, in 1972; the International Society of Paediatric Oncology, SIOP, in 1975, or the German 'Cooperative Weichteil-Sarkom-Studien', CWS, in 1981). The therapy of choice for RMS, according to the CWS, is chemotherapy because very often a good remission of the tumor is achieved. The main role of surgery is first to obtain biopsy specimens for histological diagnosis, and second aim is the complete resection of the primary and resectable metastases, for example by a neck dissection. Severe mutilation by radical surgery, especially in childhood, should be avoided in favor of multidrug chemotherapy and radiation in the interest of patient well-being. Patients: Our retrospective study shows the treatment and results of 11 patients with a rhabdomyosarcoma of the head and neck within 25 years (1972-1997). Results: Five of the 11 patients, who were treated in the years 1972-77, died (average 13 months, range 3-72 months). Five of the six patients, who were treated since 1991, have survived to date, one had a relapse of the tumor. Four special cases of patients are discussed in greater detail. Conclusion: Although the achieved mean survival time is encouraging nowadays (a 5-year survival rate of approx. 70% for all RMS is described) fatal cases are typical for this malignancy. Early diagnosis, localization, histological subtype, and prompt initiation of adequate treatment is decisive for a successful outcome.