Klinikum Stuttgart-Olgahospital, Department of Pediatric Surgery, Stuttgart, Germany.
Pediatr Blood Cancer. 2011 May;56(5):771-6. doi: 10.1002/pbc.22864. Epub 2010 Dec 15.
Solitary skeletal osteosarcoma (OS) manifestations distant from the site of the primary tumor rarely arise as only sign of disease recurrence.
This report reviews 38 patients with high-grade central OS of the limbs or axial skeleton and initial complete surgical remission (CR) who developed first recurrences as solitary osseous lesions distant from the primary tumor. The Cooperative Osteosarcoma Study Group (COSS) database was used to evaluate patient-, tumor-, and treatment-related variables and outcomes.
Thirty-eight patients (27 males and 11 females; 36 limb and 2 axial primaries) developed solitary osseous recurrences a median of 2.1 years (range: 0.5-14.3) from primary diagnosis. Relapses involved axial (24), extremity (10), or craniofacial bones (4). Treatment for recurrence included surgery (28), radiotherapy (10), and chemotherapy (27). After a median follow-up of 1.9 years (range: 0.1-21.2) from first recurrence for all 38 patients and 5.5 years (0.3-21.2) for 16 survivors (10 in continuous second CR), 2- and 5-year overall and event-free survival (EFS) probabilities were 55% and 34% and 34% and 27%, respectively. A long interval to recurrence (>1.5 years) predicted for better overall (P < 0.001) and EFS (P = 0.005). For 21 patients achieving a second CR, 2- and 5-year overall and EFS probabilities were 81% and 61% and 52% and 49%, respectively, while only 1/17 others survived beyond 5 years (P < 0.001). Survivors (14/16) had also received second-line chemotherapy.
First solitary skeletal recurrences of OS should be treated with curative intent. Some presumed bone metastases may represent second primary OSs.
孤立性骨骨肉瘤（OS）的表现远离原发性肿瘤，很少作为疾病复发的唯一迹象出现。
本报告回顾了 38 例肢体或轴骨高级别中央 OS 患者，初始完全手术缓解（CR），首次复发时表现为远离原发性肿瘤的孤立性骨病变。使用协作骨肉瘤研究组（COSS）数据库评估患者、肿瘤和治疗相关变量以及结果。
38 例患者（27 名男性和 11 名女性；36 例肢体和 2 例轴性原发性）在原发性诊断后中位时间 2.1 年（范围：0.5-14.3）出现孤立性骨骼复发。复发累及轴骨（24 例）、肢体（10 例）或颅面骨（4 例）。复发的治疗包括手术（28 例）、放疗（10 例）和化疗（27 例）。所有 38 例患者从首次复发开始的中位随访时间为 1.9 年（范围：0.1-21.2），16 例幸存者（10 例持续第二次 CR）的中位随访时间为 5.5 年（0.3-21.2），2 年和 5 年的总生存率和无事件生存率（EFS）概率分别为 55%和 34%以及 34%和 27%。复发间隔较长（>1.5 年）预测总生存率（P<0.001）和 EFS（P=0.005）更好。21 例获得第二次 CR 的患者的 2 年和 5 年总生存率和 EFS 概率分别为 81%和 61%以及 52%和 49%，而其余 17 例患者中只有 1 例超过 5 年存活（P<0.001）。幸存者（14/16）还接受了二线化疗。
首次孤立性骨骼骨肉瘤复发应采用治愈性意图进行治疗。一些假定的骨转移可能代表第二原发性骨肉瘤。
