Jürgensen O A, Reinartz G, Pichler E
Padiatr Padol. 1982;17(2):329-39.
From March 1973 to December 1981 embryonal rhabdomyosarcoma of the orbit was diagnosed in 5 children. In two children exenteration was done after a previous local radiotherapy. In one child the exenteration of the orbit was done after a primary chemo- and radiotherapy because of local progression of the tumor. In two children the exenteration was avoidable by this therapy. In four of these five children this was followed by vincristine, actinomycin D and cyclophosphamide for 16 to 24 months. After the diagnosis these four children survived until now from 3 1/2 to more than 8 years. They are free of treatment and free of disease. Seven months after diagnosis one child died with an acute respiratory distress syndrome (at post mortem: hyaline membranes). In patients with rhabdomyosarcoma of the orbit it is justified to avoid the mutilating exenteration by primary chemotherapy followed by irradiation of the reduced manner. If exenteration becomes subsequently necessary the chance for survival is not diminished in our opinion.
1973年3月至1981年12月期间,5名儿童被诊断患有眼眶胚胎性横纹肌肉瘤。两名儿童在先前接受局部放疗后进行了眼眶内容剜除术。一名儿童因肿瘤局部进展,在接受初次化疗和放疗后进行了眼眶内容剜除术。两名儿童通过这种治疗避免了眼眶内容剜除术。这5名儿童中有4名随后接受了长春新碱、放线菌素D和环磷酰胺治疗16至24个月。诊断后,这4名儿童至今存活了3年半至8年多。他们无需治疗且没有疾病。诊断后7个月,一名儿童死于急性呼吸窘迫综合征(尸检显示:透明膜)。对于眼眶横纹肌肉瘤患者,先进行化疗然后以减少剂量的方式进行放疗,以此避免致残性的眼眶内容剜除术是合理的。我们认为,如果随后有必要进行眼眶内容剜除术,生存机会并不会减少。