Parosteal osteogenic sarcoma.

A clinicopathologic study of 79 patients with parosteal osteogenic sarcoma revealed that more females than males were affected and that most of the patients were in the second to the fourth decades of life. Sixty-eight percent of the patients had involvement of the posterior aspect of the lower femoral shaft. The roentgenogram characteristically showed a large, dense lobulated mass attached by a broad base to the underlying bone but with no involvement of bone itself. Microscopically, the tumor presented as well-formed bands of osteoid within a hypocellular spindle cell stroma. Seven of the lesions had foci of high-grade osteogenic sarcoma within an otherwise typical parosteal osteogenic sarcoma. Six of the 79 lesions showed involvement of the medullary cavity at surgery. Thirty-one patients had excision as their initial treatment; four of the 31 required nv further therapy. Ten patients had resection initially; three of the ten had recurrence. Only four of 27 patients who underwent amputation initially developed pulmonary metastasis. The presence of histologically "active" tumor and medullary involvement seemed to affect the prognosis adversely. Our data indicate that complete radical removal of the tumor is the treatment of choice, with resection when feasible and amputation when necessary.     

Periosteal osteogenic sarcoma.

A study of 102 osteogenic sarcomas of the bone surface revealed that 79 were parosteal osteogenic sarcomas. Roentgenographically, these 79 were dense, lobulated lesions attached by a broad base to underlying bone, usually the lower femoral shaft. Histologically, they were low-grade osteosarcomas. The other 23 lesions (periosteal osteogenic sarcoma) usually involved the upper tibial shaft and presented as small radiolucent lesions on the surface, with formation of spicules of bone perpendicular to the bone shaft. Histologically, these 23 were relatively high-grade, predominantly chondroblastic osteogenic sarcomas. Thirteen of the 23 patients were males, and most were in the second decade of life. Of five patients who had excision of the tumor, two had recurrence. Seven of 13 patients who underwent amputation initially were alive without disease at last follow-up. Only 4 of the 23 patients have dies of metastatic disease.     

Telangiectatic osteogenic sarcoma.

Of approximately 1,999 cases of osteogenic sarcomas at the Mayo Clinic, 25 were diagnosed as telangiectatic osteogenic sarcomas. Of the 25 patients involved, 16 were males and 9 were females, and their ages ranged from 6 to 49 years. Six patients had had pathologic fracture. The lesions were typically located centrally and usually in the distal femur or proximal humerus and roentgenographically were large and purely lytic with destruction of cortex. Grossly, the lesions were cystic and contained clotted blood. Histologically, cystic spaces that contained blood were lined with anaplastic spindle cells and benign giant cells; sometimes, there were so few malignant cells that diagnosis was difficult. Usually, fine, lacelike osteoid was present. Of the 25 patients, 23 have died of metastatic disease, and another has developed pulmonary metastasis 11 months after amputation. Only one patient has survived for more than five years; however, he has developed pneumothorax. Data from this series suggest that the outlook in telangiectatic osteogenic sarcoma is more bleak than in conventional osteosarcoma.     

Biology of osteogenic sarcoma

Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. Despite significant clinical improvements over the past several decades through the use of combination chemotherapy and surgery, patients with metastatic or recurrent disease continue to have a very poor prognosis. Therefore, there is a continued need to study and understand the basic biology of osteosarcoma in order to devise more targeted and rational therapeutic strategies and ultimately to improve survival for these patients. This article reviews several aspects of osteosarcoma biology where data exist to suggest that specific pathways may play a role in the pathogenesis of this tumor. These areas include host genetic predispositions, tumor cytogenetics, molecular genetics (including the Rb, p53, RECQ helicase, and telomere pathways), and metastatic factors (ezrin, annexin 2, chemokine receptor 4, Fas/FasL pathways) that may contribute to both the initiation and the progression of tumor formation. Understanding the mechanisms of and interactions between the various molecular pathways that play a role in osteosarcoma pathogenesis may eventually lead to a more rational strategy for devising therapies targeted specifically toward these pathways.     

Management of osteogenic sarcoma patients

The experience in the management of 246 patients with local osteogenic sarcoma and 67 patients with pulmonary metastases in the All-Union Oncologic Research Center, AMS USSR, is presented here. All the patients underwent surgery, but starting in 1974, various modalities of adjuvant chemotherapy (not randomized) were applied in addition. In the surgery alone group, prognosis was very poor: only 7.0% of patients survived free of disease 5 years from the primary tumor treatment. Adjuvant chemotherapy (adriamycin + vincristine + melphalan + cyclophosphamide) following amputations increased this rate to 34.0%; combining segmental resections of an affected bone with preoperative intraarterial adriamycin infusion and radiation (36 Gy) increased the rate to 35.5%. In patients with grade 4 tumor damage (tumor cells are not found upon examination of a large number of sections), the rate increased to 57.9% (P less than .05). Another regimen of adjuvant chemotherapy (platidiam or cisplatin + adriamycin + cyclophosphamide) gives a chance to 78.8% of patients to survive 1 year free of metastases. The same chemotherapy regimen enables us to achieve an objective effect in 30.8% of patients with pulmonary metastases, and its combination with surgical metastasis ablation makes it possible to obtain a complete remission, lasting from 2 to 46 months (average 13.9 months). Toxic manifestations of the chemotherapy regimens considered are moderate. Prognosis in adjuvant chemotherapy is related to age, tumor site, its local dissemination, and morphologic type of osteogenic sarcoma.     

多发性骨肉瘤三例

目的 探讨多发性骨肉瘤的临床特点及诊断治疗的方法。方法 对3例多发性成骨肉瘤病人，进行了临床特点及治疗方法的分析，3例年龄分别为17、19、63岁；例1：病人以病变部位在右股骨远端，右肱骨近端及右胫骨近端、左锁骨、左5、6、8前肋，胸7、12椎体、左耻骨、左股骨头，以右膝关节肿痛、功能障碍就诊，例2：病人病变发生在右股骨远端，右胫骨近端，以右膝关节肿胀疼痛为主因就诊，例3：女病人病变发生在左股骨远端、左侧骨盆、右第3、5前侧肋骨，以左膝关节肿痛，左髋酸胀不适就诊。所有病人X光检查见成骨改变为主合并溶骨性改变，边界不清，骨皮质破坏，可见骨膜反应呈日光状，还有Codman三角的出现，周围见软组织阴影，其基质可见钙化，CT扫描见骨皮质破坏，已侵袭到髓腔及周围的软组织，ECT检查可见多个部位的放射性浓聚，MRI检查见病变区的信号改变，T1呈低信号影、1、2加权肿瘤呈高信号影；病理穿刺活检均可见典型的恶性梭形成骨细胞瘤的组织特征。明确诊断后均给予化疗2例男性病人并行外科手术治疗。结果3例病人均获得随访，1例死于诊断后5个月，1例死于诊断后的11个月，1例死于诊断后13个月。结论 多发性的骨肉瘤是骨肉瘤中一种十分少见的类型，治疗总的效果不理想，化疗早期有明显的局部反应，但随着化疗时间的延长发现肺转移而死亡，这可能和中后期出现的耐药反应有关，但我们可以从化疗的早期效果中看到本病有潜在的治疗意义。     

Hypofractionated accelerated radiotherapy in osteogenic sarcoma.

A method of hypofractionated accelerated radiotherapy (3 weekly fractions of 6 Gy over 2 weeks to a total tumor dose of 36 Gy) was used as single modality in 14 patients with osteogenic sarcoma for palliative treatment of the primary tumor site (six cases) or skeletal metastases (15 sites). A durable response, radiologically assessed, was obtained in 17 of the 21 (81%) irradiated sites. When this irradiation modality was combined with chemotherapy, to treat patients presenting with synchronous metastases (eight cases) or refusing amputation (five cases), a radiologically assessed response was observed in 12 of 13 (92%). In no case did a local recurrence occur before surgery or death because of progressive disease elsewhere. Of the seven patients who later had to undergo ablative surgery, a 100% and 95% tumor necrosis was observed in 6 and 1, respectively. Because of intralesional resection of primary osteogenic sarcoma after preoperative chemotherapy, seven additional patients were irradiated. None recurred at the level of the primary site. Although effective in inducing remission of osteogenic sarcoma, this irradiation method produced severe damages to normal tissues in a high proportion of patients.     

Cellular immunity indices in osteogenic sarcoma]

Prior to the treatment 39 patients with osteogenic sarcoma were examined for some cell immunity indices by the skin-allergic reaction of delayed hypersensitivity (RDH) and the reaction of suppression of leucocytes migration (RSLM). As an antigen a polysaccharide fraction of osteogenic sarcoma was employed, for the control--normal bone polysaccharide fraction. In 25 patients the course of the disease was followed up for 8--14 months after the primary examination. Both RDH and RSLM were found to show no essential difference in the reactions for polysaccharide antigens isolated from osteogenic sarcoma and normal bone. In patients with a rapid growth of the primary tumor negative RSLM was noted, while RDH indices failed to show such differences. In patients without any signs of the progressing disease during 8--14 months since the moment of the examination, as a rule, positive RSLM and RDH are noted. In patients with a precipitous course of the disease 1--8 months prior to the treatment negative RSLM and RDH are more frequently observed.