Ottaviani Giulia, Jaffe Norman
Children's Cancer Hospital, The University of Texas M.D. Anderson Cancer Center, Houston, TX 77030-4009, USA.
Cancer Treat Res. 2009;152:3-13. doi: 10.1007/978-1-4419-0284-9_1.
Osteosarcoma derives from primitive bone-forming mesenchymal cells and is the most common primary bone malignancy. The incidence rates and 95% confidence intervals of osteosarcoma for all races and both sexes are 4.0 (3.5-4.6) for the range 0-14 years and 5.0 (4.6-5.6) for the range 0-19 years per year per million persons. Among childhood cancers, osteosarcoma occurs eighth in general incidence and in the following order: leukemia (30%), brain and other nervous system cancers (22.3%), neuroblastoma (7.3%), Wilms tumor (5.6%), Non-Hodgkin lymphoma (4.5%), rhabdomyosarcoma (3.1%), retinoblastoma (2.8%), osteosarcoma (2.4%), and Ewing sarcoma (1.4%). The incidence rates of childhood and adolescent osteosarcoma with 95% confidence intervals areas follows: Blacks, 6.8/year/million; Hispanics, 6.5/year/million; and Caucasians, 4.6/year/million. Osteosarcoma has a bimodal age distribution, having the first peak during adolescence and the second peak in older adulthood. The first peak is in the 10-14-year-old age group, coinciding with the pubertal growth spurt. This suggests a close relationship between the adolescent growth spurt and osteosarcoma. The second osteosarcoma peak is in adults older than 65 years of age; it is more likely to represent a second malignancy, frequently related to Paget's disease. The incidence of osteosarcoma has always been considered to be higher in males than in females, occurring at a rate of 5.4 per million persons per year in males vs. 4.0 per million in females, with a higher incidence in blacks (6.8 per million persons per year) and Hispanics (6.5 per million), than in whites (4.6 per million). Osteosarcoma commonly occurs in the long bones of the extremities near the metaphyseal growth plates. The most common sites are the femur (42%, with 75% of tumors in the distal femur), the tibia (19%, with 80% of tumors in the proximal tibia), and the humerus (10%, with 90% of tumors in the proximal humerus). Other likely locations are the skull or jaw (8%) and the pelvis (8%). Cancer deaths due to bone and joint malignant neoplasms represent 8.9% of all childhood and adolescent cancer deaths. Death rates for osteosarcoma have been declining by about 1.3% per year. The overall 5-year survival rate for osteosarcoma is 68%, without significant gender difference. The age of the patient is correlated with the survival, with the poorest survival among older patients. Complete surgical excision is important to ensure an optimum outcome. Tumor staging, presence of metastases, local recurrence, chemotherapy regimen, anatomic location, size of the tumor, and percentage of tumor cells destroyed after neoadjuvant chemotherapy have effects on the outcome.
骨肉瘤起源于原始的成骨间充质细胞，是最常见的原发性骨恶性肿瘤。所有种族和性别的骨肉瘤发病率及95%置信区间为：0至14岁年龄段为每年每百万人4.0（3.5 - 4.6）例，0至19岁年龄段为每年每百万人5.0（4.6 - 5.6）例。在儿童癌症中，骨肉瘤的总体发病率排名第八，顺序如下：白血病（30%）、脑及其他神经系统癌症（22.3%）、神经母细胞瘤（7.3%）、肾母细胞瘤（5.6%）、非霍奇金淋巴瘤（4.5%）、横纹肌肉瘤（3.1%）、视网膜母细胞瘤（2.8%）、骨肉瘤（2.4%）和尤因肉瘤（1.4%）。儿童和青少年骨肉瘤的发病率及95%置信区间如下：黑人，每年每百万人6.8例；西班牙裔，每年每百万人6.5例；白种人，每年每百万人4.6例。骨肉瘤具有双峰年龄分布，第一个高峰出现在青春期，第二个高峰出现在老年期。第一个高峰在10至14岁年龄组，与青春期生长突增相吻合。这表明青春期生长突增与骨肉瘤之间存在密切关系。骨肉瘤的第二个高峰出现在65岁以上的成年人中；它更可能代表第二种恶性肿瘤，通常与佩吉特病有关。骨肉瘤的发病率一直被认为男性高于女性，男性每年每百万人发病5.4例，而女性为每百万人4.0例，黑人（每年每百万人6.8例）和西班牙裔（每年每百万人6.5例）的发病率高于白人（每年每百万人）。骨肉瘤通常发生在四肢长骨靠近干骺端生长板的部位。最常见的部位是股骨（42%，其中75%的肿瘤位于股骨远端）、胫骨（19%，其中80%的肿瘤位于胫骨近端）和肱骨（10%，其中90%的肿瘤位于肱骨近端）。其他可能的部位是颅骨或颌骨（8%）和骨盆（8%）。骨和关节恶性肿瘤导致的癌症死亡占所有儿童和青少年癌症死亡的8.9%。骨肉瘤的死亡率每年下降约1.3%。骨肉瘤的总体5年生存率为68%，无显著性别差异。患者年龄与生存率相关，老年患者的生存率最差。完整的手术切除对于确保最佳治疗效果很重要。肿瘤分期、转移情况、局部复发、化疗方案、解剖位置、肿瘤大小以及新辅助化疗后被破坏的肿瘤细胞百分比都会影响治疗结果。
