Purpose: Osteosarcoma may arise as a secondary cancer following leukemias or lymphomas. We intended to increase the knowledge about such rare events. Patients and methods: We searched the Cooperative Osteosarcoma Study Group’s database for individuals who developed their osteosarcoma following a previous hematological malignancy. The presentation and treatment of both malignancies was investigated, and additional neoplasms were noted. Outcomes after osteosarcoma were analyzed and potential prognostic factors were searched for. Results: A total of 33 eligible patients were identified (male: 23, female: 10; median age: 12.9 years at diagnosis of hematological cancer; 20 lymphomas, 13 leukemias). A cancer predisposition syndrome was evident in one patient only. The hematological cancers had been treated by radiotherapy in 28 (1 unknown) and chemotherapy in 26 cases, including bone-marrow transplantation in 9. The secondary bone sarcomas (high-grade central 27, periosteal 2, extra-osseous 2, undifferentiated pleomorphic sarcoma of bone 2) arose after a median lag-time of 9.4 years, when patients were a median of 19.1 years old. Tumors were considered radiation-related in 26 cases (1 unknown). Osteosarcoma-sites were in the extremities (19), trunk (12), or head and neck (2). Metastases at diagnosis affected eight patients. Information on osteosarcoma therapy was available for 31 cases. All of these received chemotherapy. Local therapy involved surgery in 27 patients, with a good response reported for 9/18 eligible patients. Local radiotherapy was given to three patients. The median follow-up was 3.9 (0.3–12.0) years after bone tumor diagnosis. During this period, 21 patients had developed events as defined, and 15 had died, resulting in 5-year event-free and overall survival rates of 40% (standard error: 9%) and 56% (10%), respectively. There were multiple instances of additional neoplasms. Several factors were found to be of prognostic value (p< 0.05) for event-free (osteosarcoma site in the extremities) or overall (achievement of a surgical osteosarcoma-remission, receiving chemotherapy for the hematologic malignancy) survival. Conclusions: We were able to prove radiation therapy for hematological malignancies to be the predominant risk factor for later osteosarcomas. A resulting overrepresentation of axial and a tendency towards additional neoplasms affects prognosis. Still, selected patients may become long-term survivors with appropriate therapies, which is an argument against therapeutic negligence.
目的：骨肉瘤可作为白血病或淋巴瘤后的继发性癌症发生。本研究旨在增进对此类罕见事件的认识。患者与方法：我们在合作骨肉瘤研究组数据库中检索了既往患有血液系统恶性肿瘤后发生骨肉瘤的病例。调查了两种恶性肿瘤的表现与治疗情况，并记录了其他肿瘤事件。分析了骨肉瘤后的预后结果，并探索了潜在的预后因素。结果：共纳入33例符合条件的患者（男性23例，女性10例；血液肿瘤诊断中位年龄12.9岁；淋巴瘤20例，白血病13例）。仅1例患者存在明确的癌症易感综合征。血液系统恶性肿瘤的治疗包括：28例接受放疗（1例不详），26例接受化疗，其中9例进行了骨髓移植。继发性骨肉瘤（高级别中央型27例、骨膜型2例、骨外型2例、骨未分化多形性肉瘤2例）的中位潜伏期为9.4年，诊断时患者中位年龄19.1岁。26例（1例不详）被判定为放疗相关肿瘤。骨肉瘤部位分布于四肢（19例）、躯干（12例）及头颈部（2例）。8例患者在诊断时已发生转移。31例患者可获得骨肉瘤治疗信息，所有患者均接受化疗。27例患者接受手术局部治疗，其中9/18例符合条件的患者报告良好反应。3例患者接受局部放疗。骨肿瘤诊断后的中位随访时间为3.9年（范围0.3-12.0年）。随访期间，21例患者发生既定事件，15例死亡，5年无事件生存率和总生存率分别为40%（标准误9%）和56%（标准误10%）。多例患者出现其他肿瘤事件。研究发现多个因素对无事件生存（骨肉瘤位于四肢）或总生存（实现骨肉瘤手术缓解、曾接受血液恶性肿瘤化疗）具有预后价值（p<0.05）。结论：本研究证实血液系统恶性肿瘤的放射治疗是继发骨肉瘤的主要危险因素。由此导致的躯干部位肿瘤高发及其他肿瘤发生倾向会影响预后。尽管如此，经过恰当治疗，部分患者仍可获得长期生存，这为反对治疗疏忽提供了依据。
肿瘤(癌症)患者之家