Seidl R O, Gerein V, Vogel H J, Ernst A
Klinik für Hals-, Nasen-, Ohrenheilkunde, Kopf- und Halschirurgie, Unfallkrankenhaus Berlin, Warenerstrasse 7, 12683 Berlin.
HNO. 2001 Sep;49(9):744-9. doi: 10.1007/s001060170047.
A 13-year-old girl suffered from a mesenchymal chondrosarcoma of the left maxilla. The therapeutic options and the prognosis for this disease are described with respect to the currently known 72 cases in the literature. Mesenchymal chondrosarcomas are rare tumors of the bone and soft tissue. The first clinical symptom is a painless swelling of the facial skull. They occur largely in the 2nd and 3rd decades of life, preferentially in males. Radiological criteria for the identification of this type of tumor include focal ossification areas which are accompanied by non-calcified regions. Complete surgical removal of the tumor is the therapy of choice. Pre- and postoperative chemotherapy can have a beneficial effect. The final outcome of the disease is difficult to evaluate since late complications (e.g., reoccurrence and/or metastases) appear even after 20 years and only a small number of cases have been reported. At present, the 5-year survival rate is reported to be 54-82% and the 10-year rate 28-56%.
一名13岁女孩患有左上颌骨间叶性软骨肉瘤。本文结合文献中目前已知的72例病例,描述了该疾病的治疗选择和预后情况。间叶性软骨肉瘤是一种罕见的骨与软组织肿瘤。首发临床症状为面颅骨无痛性肿胀。该肿瘤主要发生于20至30岁人群,男性更为多见。识别此类肿瘤的放射学标准包括伴有非钙化区域的局灶性骨化区。肿瘤的完整手术切除是首选治疗方法。术前和术后化疗可能会产生有益效果。由于即使在20年后仍会出现晚期并发症(如复发和/或转移),且仅有少数病例报道,因此该病的最终结局难以评估。目前,据报道5年生存率为54% - 82%,10年生存率为28% - 56%。