Hess A, Schröder U, Schröder R, Michel O
Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde.
Laryngorhinootologie. 1998 Oct;77(10):557-63. doi: 10.1055/s-2007-997026.
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 ubiquitiously. 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.
Our retrospective study shows the treatment and results of 11 patients with a rhabdomyosarcoma of the head and neck within 25 years (1972-1997).
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.
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.
软组织肉瘤约占14岁以下儿童所有肿瘤的7%。其中最常见的类型（约50%）是横纹肌肉瘤（RMS）。在约占所有RMS 35%的头颈部，该肿瘤可在多处发现。常见的发病部位是眼眶、鼻窦以及脸颊和颈部的软组织。RMS的预后以前很差，但自20世纪70年代确立多模式治疗以来（例如1972年的横纹肌肉瘤协作组研究，IRS；1975年的国际小儿肿瘤学会，SIOP；或1981年德国的“软组织肉瘤协作研究”，CWS）有了显著改善。根据CWS的研究，RMS的首选治疗方法是化疗，因为通常能使肿瘤得到良好缓解。手术的主要作用首先是获取活检标本进行组织学诊断，其次是彻底切除原发灶和可切除的转移灶，例如通过颈部清扫术。为了患者的健康，应避免根治性手术造成严重毁容，尤其是在儿童期，而应选择多药化疗和放疗。
我们的回顾性研究展示了25年（1972 - 1997年）内11例头颈部横纹肌肉瘤患者的治疗情况及结果。
在1972 - 1977年接受治疗的11例患者中有5例死亡（平均13个月，范围3 - 72个月）。自1991年以来接受治疗的6例患者中有5例至今存活，1例肿瘤复发。详细讨论了4例特殊患者情况。
尽管如今所达到的平均生存时间令人鼓舞（据描述所有RMS的5年生存率约为70%），但这种恶性肿瘤仍有典型的致命病例。早期诊断、定位、组织学亚型以及及时开始适当治疗对于成功治疗结果至关重要。
