Tebbi C K, Gaeta J
Department of Pediatrics, Roswell Park Memorial Institute, Buffalo, New York 14263.
Pediatr Ann. 1988 Apr;17(4):285-300. doi: 10.3928/0090-4481-19880401-08.
Osteosarcoma is the most common bone tumor of children and adolescents. The peak incidence of the disease is in the 15 to 19 year age group. The disease is more commonly seen in males than females. While several factors, including exposure to radiation, genetic disorders such as retinoblastoma, and high rate of bone growth, have been associated with osteosarcoma, in most cases no definite etiology can be established. Osteosarcoma usually originates in the metaphyseal region of long bones and extends through the cortex, causing varying degrees of bone destruction and expansion of periosteum. The radiographic appearance caused by this process is often referred to as "sun burst" sign. Positive diagnosis of osteosarcoma is made by histopathology. The histopathological classification of osteosarcoma can also predict the degree of aggressive behavior of this tumor and thus has prognostic significance. Surgery, including amputation or limb-salvage procedure, is the mainstay of treatment of osteosarcoma. It is now unequivocally established that adjuvant chemotherapy will prolong the survival of patients with this disease. Chemotherapy agents often used include platinum derivates, methotrexate, vincristine, cyclophosphamide, adriamycin, actinomycin D, bleomycin and DTIC. Depending on surgical decision, these agents can be used prior to or after the operation. Immediate fitting with prosthesis and provision of appropriate medical and psychological support in the care of these patients is essential.
骨肉瘤是儿童和青少年中最常见的骨肿瘤。该疾病的发病高峰在15至19岁年龄组。男性比女性更易患此病。虽然包括接触辐射、视网膜母细胞瘤等遗传疾病以及高骨生长率等多种因素与骨肉瘤有关,但在大多数情况下无法确定明确的病因。骨肉瘤通常起源于长骨的干骺端区域,并穿过皮质,导致不同程度的骨质破坏和骨膜增生。这一过程所导致的影像学表现通常被称为“日光放射”征。骨肉瘤的确诊依靠组织病理学检查。骨肉瘤的组织病理学分类也能够预测该肿瘤的侵袭性程度,因而具有预后意义。手术,包括截肢或保肢手术,是骨肉瘤治疗的主要手段。现已明确证实,辅助化疗可延长该疾病患者的生存期。常用的化疗药物包括铂衍生物、甲氨蝶呤、长春新碱、环磷酰胺、阿霉素、放线菌素D、博来霉素和达卡巴嗪。根据手术决策,这些药物可在手术前或手术后使用。对于这些患者,立即安装假肢并提供适当的医疗和心理支持至关重要。