A modular endoprosthetic system, noninvasively extendable, for young patients with osteosarcoma.
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We have studied the results of reconstruction of a large skeletal defect using a vascularized fibula graft in 62 consecutive cases. We were particularly interested in factors that may significantly influence the outcome of union. In at least 90% of cases, eventual union can be expected in the reconstruction of large skeletal defects resulting from tumour resection, traumatic bone loss, or nonunion. The results of reconstruction for osteomyelitis were less favorable. Statistical analysis of the influences of bone graft polarity, internal fixation, additional bone graft polarity, internal fixation, additional bone graft material, and the length of the graft on the outcome of union revealed that the use of stable fixation and additional bone graft material significantly enhanced bone union, whereas the length or polarity of the graft had no influence.
A rare case of Ewing's sarcoma metastatic to the cerebellum is presented. Neurosurgical intervention was required which played a significant role in the treatment of this patient. The incidence and treatment of central nervous system involvement from Ewing's sarcoma is reviewed and discussed.
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Two patients with osteosarcoma of a lower limb and presumed lymph node metastases are described. The metastases presented in both children as palpable masses, one in the inguinal region and the other in the pelvis. One was visible on plain radiography; both were demonstrated on computed tomography and bone scintigraphy. The inguinal mass was present at the first examination and was resected with the primary lesion. The pelvic mass presented 12 months after amputation and was unresectable; its subsequent rapid growth was demonstrated by computed tomography. In one patient the osteosarcoma was of the osteoblastic type.
Desmoplastic fibroma of bone is a rare benign tumor, first described by Jaffe in 1958. Since then approximately 80 cases have been reported in the long bones, although the tumor also involves the jaw bones, particularly the mandible. The plain film features consist of a lytic, expansile lesion, usually in the metaphysis of bone, sometimes extending into the soft tissues. We present two cases involving the long bones in which the correct diagnosis was suggested by computed tomography (CT). The CT appearance of the lesions is described.
Three cases featuring the coincidence of 2 different primary bone tumors at the same site are presented. In 2 cases, the combination was not one of giant cell tumor and enchondroma, in the 3rd one, of giant cell tumor and non-ossifying fibroma. The patho-genetical and prognostical problems of these cases are discussed.
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A femur bone metastasis from breast carcinoma was treated by curetting and filling with acrylic cement and osteosynthesis. The histological study of the resected proximal third of the femur five months after surgery shows a thin layer of connective tissue between bone and cement. There are no neoplastic cells in this connective tissue nor in the spongious bone of the proximal femur. Instead the diaphyseal channel is fully invaded with neoplastic cells. Pathogenesis and validity of surgical treatment by the emptying and filling with acrylic cement of bone metastases are discussed.
A 25-year-old patient with osteosarcoma of the right distal femur underwent a bone scintigraphy with Tc-99m methylene diphosphonate (MDP). Whole-body bone scan revealed extensive metastatic disease in the abdominal region. Abdominal computerized tomography confirmed the presence of ascites and calcified masses on the greater omentum and peritoneal surfaces. Here we describe a case of unusual metastatic pattern of an osteosarcoma showing extensive intraabdominal metastases without prominent lung involvement after intensive chemotherapy.
We treated a 26-year-old man with a 19-year history of Ollier's disease. Secondary chondrosarcomas developed metachronously at four separate locations: both femora, left proximal tibia and fibular head. All four lesions were surgically excised, and each specimen was histologically identified as grade 1 or 2 chondrosarcoma. Clinical follow-up for 20 years beginning at the time of first tumor surgery has shown no evidence of local recurrence or metastasis. This is the first report of multiple bilateral metachronous malignant transformation of multiple chondromatoses in a patient with Ollier's disease.
Multidrug resistance (MDR), mediated by P-glycoprotein (P-gp), is an in vitro phenomenon observed within tumour cells, suggesting cross-resistance to unrelated drugs, and expression of P-gp may therefore affect the prognosis and incidence of recurrence after treatment. The mutant p53 protein causes reduced tumour suppression. Co-expression of p53 and P-gp is related to short survival, increased tumour activity and drug resistance. The purpose of this study was to measure the expression of p53 protein and P-gp in osteosarcoma tissue and assess its prognostic significance. Fifty-two tumour specimens were evaluated. The correlation between p53 and P-gp expression was significant (P=0.0008). In univariate analysis of survival, p53 protein was not significant (P=0.2) but P-gp was significant (P=0.0001). The co-expression of p53 and P-gp was the strongest indicator of a short survival according to multivariate analysis (P=0.0004).
Clear cell chondrosarcoma is a rare variant of the bone tumors with distinct radiologic and pathologic features. In this report two cases of clear cell chondrosarcomas with atypical radiologic appearances are presented. The findings of X-ray films and MRI are described with histologic correlation. In the first case the lesion showed a very expansile and long segment involvement of the humerus. In the second case the lesion was located in the diaphysis of the femur causing a large cortical destruction.
We performed five reconstructions by bone transport in patients with bone sarcoma: three osteosarcomas, one Ewing's sarcoma and one chondrosarcoma. Four sarcomas were in the distal femur and one in the shaft of the tibia. Four patients received multidrug chemotherapy. The average length of the skeletal defect after tumour resection was 17 (range 10-25) cm. All patients underwent double elongation from both proximal and distal sites of the bone defect. The average follow-up period was 48 (range 40-66) months, and the average duration of external fixation was 32 months (range 579-1340 days). In one case, bone formation was satisfactory, but in the other cases, it was poor and slow. The average treatment index was 95 (range 53-191) days per 1 cm of elongation. In one case, the bone fragment disappeared during the bone transport. In one case, the end of the bone protruded from the skin, and osteomyelitis set in. This patient underwent above-knee amputation due to failure of infection control. Three patients suffered talipes equinus. One patient died of pulmonary metastasis. Two patients had fair and three had poor function. This method is not recommended for patients with bone sarcoma who may have a poor prognosis, as it has an unacceptably high complication rate.
Osteosarcoma was detected in two siblings. Their human leukocyte antigen (HLA) phenotypes were completely identical, although they were different from those of osteosarcoma patients in previous reports. Despite an extensive search of family and past history, no significant background related to the induction of cancer could be found. These cases suggest that genetic similarity may influence the development of osteosarcoma. Ascertainment of the HLA phenotypes in siblings with osteosarcoma might be a useful strategy to facilitate the early diagnosis of this tumor.
The potential role of thallium-201 (201Tl) scintigraphy in the imaging of various musculoskeletal tumors was investigated in 80 patients. Uptake of 201Tl was observed in 17 (100%) malignant bone tumors, 6 (100%) giant cell tumors, and 2 of 8 (25%) benign bone tumors. Nineteen of 30 malignant soft tissue tumors (63%) were positive for 201Tl scintigraphy, while 2 of 14 benign soft tissue tumors (14%) yielded positive results. None of 6 liposarcomas were visualized by 201Tl scintigraphy. There was no 201Tl uptake in the tissues of 5 non-tumorous conditions. Ten patients with osteosarcoma were evaluated by 201Tl scintigraphy both pre- and post-chemotherapy. There was a significant correlation between changes in tumor-to-normal count ratio and percent necrosis of the resected tumor. The mean decrease in tumor-to-normal count ratio was 71% for patients with >/=90% tumor necrosis and 26% for those with <90% tumor necrosis. Serial 201Tl scintigraphy, with quantitative analysis of alterations in 201Tl uptake, may provide a quantitative and objective measure of the effect of preoperative chemotherapy in patients with malignant bone tumors.
The ultrasonographic appearances of osteosarcomas and the roles of ultrasonography in the diagnosis and surgical staging of osteosarcomas were investigated. A comparative study was performed on 45 cases of osteosarcomas by ultrasonography and radiography. Bony changes, periosteal reaction and soft tissue mass were evaluated for each lesion. The results showed that ultrasonography revealed a solid mass around bone in 42 patients, bone destruction in 24 patients and periosteal reaction in 16 patients. Plain radiographs showed bony changes in 44 patients and no bony change in remaining one patient, shadowing of soft tissue swelling in 30 patients, and pulmonary metastases in 3 patients. Surgical biopsy and pathological examination confirmed osteosarcoma in all 45 patients. Soft tissue mass was confirmed in 42 patients surgically. The diagnostic accuracy of soft tissue masses by ultrasonography and radiography was 100 % (42/42) and 71.4 % (30/42), respectively. The positive rate of ultrasonography and radiography in displaying bony changes was 53.3 % (24/45) and 97.8 % (44/45), respectively. In conclusion, in the detection of soft tissue mass of osteosarcoma, ultrasonography is superior to radiography, and in displaying bony changes of osteosarcomas, radiography is superior to ultrasonography. So it may come to a conclusion that plain radiography combined with ultrasonography can completely display the bony and soft tissue lesion of osteosarcomas.