Brandal Petter, Bjerkehagen Bodil, Bruland Oyvind S, Skjeldal Sigmund, Bogsrud Trond V, Hall Kirsten S
Department of Oncology, Division of Cancer Medicine and Radiotherapy, The Norwegian Radium Hospital, Montebello, Oslo, Norway.
Acta Oncol. 2009;48(8):1165-72. doi: 10.3109/02841860903032809.
Background. The purpose of this work was to study clinical and histopathological tumor characteristics of patients treated for synchronous or metachronous skeletal osteosarcoma at The Norwegian Radium Hospital from January 1, 1980 to January 1, 2008. Patients and methods. The hospital sarcoma database and patient records were reviewed to identify cases with synchronous or metachronous skeletal osteosarcoma. Patients with more than one skeletal lesion in the absence of pulmonary or other soft tissue tumor manifestations were included in the study, and histopathological slides from these tumors were reviewed. Results. Among a total of 297 registered osteosarcoma patients, six with synchronous (2.0%) and 10 with metachronous (3.4%) skeletal osteosarcomas were identified. All tumors were of high-grade malignancy. Treatment at the time of the first osteosarcoma diagnosis was in most cases wide resections and multi-agent chemotherapy according to international protocols, whereas the treatment for metachronous tumors was individualized and in general much less intensive. One patient was diagnosed with Li-Fraumeni syndrome, two other individuals may be suspected to have the same syndrome, and yet another patient had previously been treated for a bilateral retinoblastoma. Thirteen patients are dead, 11 from metastatic osteosarcoma, one from myelodysplastic syndrome, and one from wound infection and methotrexate-related nephrotoxicity; whereas three patients are still alive with no evidence of osteosarcoma. Conclusions. The prognosis for patients with synchronous and metachronous skeletal osteosarcoma is poor. However, because long-term survival is seen, aggressive treatment to selected cases, e.g., patients with an osteosarcoma predisposing syndrome and/or late occurring metachronous tumours, is justified. Revealing a possible clonal relationship between these tumors, e.g., by karyotyping, may be of interest for estimating prognosis and guide therapy intensiveness.
背景。本研究旨在探讨1980年1月1日至2008年1月1日期间在挪威镭医院接受同步或异时性骨骼骨肉瘤治疗的患者的临床和组织病理学肿瘤特征。患者与方法。回顾医院肉瘤数据库和患者记录，以确定同步或异时性骨骼骨肉瘤病例。纳入研究的患者为在无肺或其他软组织肿瘤表现的情况下有一个以上骨骼病变的患者，并对这些肿瘤的组织病理学切片进行了复查。结果。在总共297例登记的骨肉瘤患者中，确定了6例同步性（2.0%）和10例异时性（3.4%）骨骼骨肉瘤患者。所有肿瘤均为高级别恶性肿瘤。首次骨肉瘤诊断时的治疗在大多数情况下是根据国际方案进行广泛切除和多药化疗，而异时性肿瘤的治疗是个体化的，总体上强度要小得多。一名患者被诊断为李-弗劳梅尼综合征，另外两名个体可能被怀疑患有相同综合征，还有一名患者曾接受双侧视网膜母细胞瘤治疗。13例患者死亡，11例死于转移性骨肉瘤，1例死于骨髓增生异常综合征，1例死于伤口感染和甲氨蝶呤相关的肾毒性；而3例患者仍然存活，无骨肉瘤证据。结论。同步和异时性骨骼骨肉瘤患者的预后较差。然而，由于观察到长期生存情况，对选定病例，如患有骨肉瘤易感综合征和/或晚期发生的异时性肿瘤的患者进行积极治疗是合理的。通过核型分析等方法揭示这些肿瘤之间可能的克隆关系，可能有助于估计预后并指导治疗强度。
