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Response of osteosarcoma and Ewing sarcoma to chemotherapy: imaging evaluation.

The importance of diagnostic imaging in evaluating therapeutic response of bone sarcomas has increased with the use of chemotherapy administered before radiation therapy or surgery. The information provided by imaging studies is used to estimate prognosis; modify preoperative chemotherapy; and plan radiation therapy, surgery, and postoperative chemotherapy. This review examines the value and illustrates some pitfalls of traditional imaging methods, including conventional radiography, angiography, CT, and radionuclide scintigraphy, and discusses recent efforts to monitor therapy by using MR imaging and MR spectroscopy.

Angiography↗

Limb reconstruction with the callus distraction method after bone tumor resection.

The callus distraction method was applied to nine patients who were referred to us because of a bone tumor. Their mean age was 17 years and 3 months (range 7-37 years). Three were female, and 6 were male. All of the tumors were localised on the femur, and the histological diagnosis was two chondrosarcomas, one Ewing's sarcoma, three osteosarcomas, one giant cell bone tumor, and the remainder benign fibrous histiocytoma. The mean length of the defect after resection of the tumor was 11.5 (range 8-20) cm. Preoperative and postoperative chemotherapy were applied to patients with osteosarcoma and Ewing's sarcoma. The patients were followed up for 22 (range 15-30) months on average. The mean period of use of the external fixator was 12.5 (range 8-18) months. One patient suffered a tumour recurrence and died after 20 months. Complications included one deep infection, one skin invagination, and one premature consolidation and bone bridge in the defect area. All of the complications were successfully treated. Functional evaluation gave excellent results in four patients, good in three, and fair in two. This method can be used without any need for massive autogenous bone graft in repairing defects of any length and diameter produced after excision of the lesion and thus can be considered as an alternative to other techniques.

Adolescent↗

[Pulmonary metastasectomy for osteosarcomas and soft tissue sarcomas].

To evaluate the efficacy of aggressive pulmonary metastasectomy for treating osteosarcomas and soft tissue sarcomas, we reviewed 105 cases treated in our hospital between 1990 and 2002. There were 57 males and 48 females, 44 osteosarcomas (OS), 21 synovial sarcomas (Syno), 16 malignant fibrous histiocytomas (MFH), 4 leiomyosarcomas (Leio), 4 alveolar soft part sarcomas (ASPS) and 16 others (including chondrosarcoma and liposarcoma). A total of 904 metastases were resected (8.6(0-49)/patients) and 244 thoracotomies (2.3/patients) were performed. Mean number of initial metastasectomies was 3.7 (0-26), disease-free interval (DFI) was 13. 8 months (0-96), and mean diameter of maximum resected metastases for one patient was 20.4 (5-90) mm. Five-and 10-year survival rates of all cases were 44.9% and 32.0% respectively. Those of OS were 45.8% and 38.5%, and those of soft tissue sarcomas were 44.2% and 25.5%, respectively. The highest 5-and 10-year survival rates among soft tissue sarcomas was attained in ASPS and Leio (75%), and others (51.6% and 38.7%), followed by Syno (42.9% and 12.4%). Long-term survival was not attained in MFH (30.9%: 5 years). Analysis of histological types of the tumors and numbers of resected pulmonary metastases showed that the largest number of metastases were resected in ASPS (16/case) and Syno (13.8/case), followed by OS (9.0/case). MFH had the largest (27.1 mm) mean diameter of maximum resected metastases, followed by Leio (27.0 mm). Analysis of prognostic factors indicated that curativity was the most important prognostic factor: curative cases 42.2% (10-year survival) vs noncurative cases 4.2% (6-year survival). Number of resected metastases and mean diameter of maximum tumor size also affected the patient survival.

Adolescent↗

[Osteosarcoma and Ewing's sarcoma. Improved recovery chances in the past 25 years according to the experience of Balgrist Hospital].

At the Balgrist hospital 38 patients with osteosarcoma and 18 patients with Ewing sarcoma were treated from 1970 through 1992; a follow-up of at least three years is available for all patients. Disease-free survival in this period of time has increased from 20% (Ewing sarcoma) and 30% (osteosarcoma) to over 75%. While systemic tumor control was improved by systematic chemotherapy, local tumor control was improved by appropriate biopsy techniques, and amputations have been replaced by local resections, in most cases with reconstruction (arthrodesis, rotation plasty, endoprosthesis, allografts), which require further improvements. With respect to cure of the neoplastic disease, the currently available methods have been used to their maximum potential, and a steady state has been reached regarding survival. Further improvement of survival will therefore depend on the introduction of completely new modalities.

Adolescent↗

[Osteosarcoma and Ewing's sarcoma--The most frequent malignant bone tumors in children--therapy and outcome].

BACKGROUND: Osteosarcoma and Ewing's sarcoma are the most frequent malignant bone tumors in children and young adults with relatively poor overall survival rates. METHODS: Between January 1980 and December 1994, 175 children with osteosarcoma and 64 children with Ewing's sarcoma were treated at the author's institution. 22 children had synchronous metastases, 19 patients had a pathologic fracture. Both groups were treated systemically with chemotherapy regimens (COSS and CESS). Local therapy was amputation or tumor resection and endoprosthetic replacement or biological reconstruction with wide or radical resection margins. In case of Ewing's sarcoma in 35 patients postoperative radiation therapy was done. RESULTS: Five-year overall survival rate for osteosarcoma and Ewing's sarcoma patients is about 63 %, ten-year survival rate for osteosarcoma patients is 60.2 %, for Ewing's sarcoma patients 54.5 %. Prognostic factors significantly influencing overall survival rates are tumor response to chemotherapy (p values = 0.0056 and 0.013, respectively), surgical treatment with adequate resection margins (p value = 0.0001 for osteosarcoma patients) and development of postoperative metastases (p value = 0.0001 for both groups). CONCLUSION: For both groups of malignant bone tumors systemic chemotherapy as well as adequate surgical therapy are necessary to reduce the rates of local recurrences and to achieve better survival rates.

Adolescent↗

Recurrent, multiple, calcified soft tissue metastases from osteogenic sarcoma without pulmonary involvement.

Osteosarcoma (osteogenic sarcoma) metastasizes primarily to the lung. With the introduction of neoadjuvant chemotherapy as part of the treatment, the overall and disease-free survival rates have dramatically improved. In this case report, a young man with multiple soft tissue and bone metastases, including a rare large bone-forming retroperitoneal metastasis, is described. Despite the extensive extrapulmonary metastases, the patient did not develop pulmonary metastases in the 4 years following initial presentation of the primary tumour.

Adult↗

Presenting symptoms and treatment delay in osteosarcoma and Ewing's sarcoma.

Presenting symptoms and treatment delay were investigated in a series of 84 osteosarcomas and 40 Ewing's sarcomas. The treatment delay averaged 6.4 months for osteosarcomas and 9.6 months for Ewing's sarcomas. In both types the delay was relatively short in patients with constant pain and swelling. The prognosis was about the same whether the delay was short or long. This may be explained by the more severe nature of the presenting symptoms, in particular the constant pain and swelling, in aggressive tumours, a factor that also tends to mask other relevant prognostic parameters. It is concluded that the presenting symptoms and the treatment delay constitute important pathophysiologic factors which should be given more attention when analysing material from bone sarcomas.

Adolescent↗

Prognosis of radiation-induced bone sarcoma is similar to primary osteosarcoma.

UNLABELLED: Radiation-induced sarcoma of bone (RISB) is thought to be associated with a poor prognosis. The purpose of this study was to determine disease-free and overall survival of patients treated for RISB, and whether there is a role for limb sparing surgery. Twenty-four patients had a mean latency of 16 years between radiation for their index cancer and RISB diagnosis. The most common tumor was osteosarcoma (n = 17). Ten patients with localized disease treated aggressively with chemotherapy and surgical resection had estimated 5-year disease-free and overall survival rates of 58% and 69% respectively. Patients treated by surgery alone or those with metastases at diagnosis had inferior outcomes. Patients who received a complete course of chemotherapy demonstrated better histologic tumor response and improved survival. There was no difference in survival between the limb sparing surgery (n = 12) or amputation (n = 8) groups. However, limb salvage patients had slightly higher rates of local tumor relapse and post-operative complications. Functional outcome following limb sparing surgery for 10 patients with RISB was similar to a matched cohort treated for primary osteosarcoma. An aggressive treatment approach for patients with RISB may provide similar rates of local recurrence and metastasis and functional outcomes compared to patients with primary osteosarcoma. LEVEL OF EVIDENCE: Therapeutic study, level IV-1.

Adolescent↗

Some retinoblastomas, osteosarcomas, and soft tissue sarcomas may share a common etiology.

DNA and RNA were extracted from primary human osteosarcomas and soft tissue sarcomas obtained from patients without retinoblastoma and were analyzed by hybridization with a cDNA probe for RB mRNA; absence or alterations of the RB gene are associated with development of retinoblastoma. Most of the osteosarcomas or soft tissue sarcomas examined by us did not express detectable levels of RB mRNA, whereas normal cells and epithelial tumor cells did. One osteosarcoma expressed a 2.4-kilobase transcript in addition to a normal 4.7-kilobase species. Our data suggest that transcriptional inactivation or post-transcriptional down-regulation of the RB gene may be important in the etiology of some osteosarcomas and soft tissue sarcomas as well as retinoblastomas.

Bone Neoplasms↗

Lymphatic dissemination of bone and soft tissue sarcomas: a lymphographic investigation.

The series consisted of 132 patients, 61 with primary bone sarcomas and 71 with primary soft tissue sarcomas. The patients were all evaluated by lymphography. The investigation included both patients who had not yet been treated and patients with suspected or confirmed metastases. All tumour diagnoses were confirmed microscopically. The findings as regards dissemination were based on clinical examinations, laboratory tests, roentgen examinations and lymphographies. In some cases, lymph node biopsies and surgical observations were also used. A total of 151 lymphographies were performed and 281 follow-up films taken. Preoperative lymphography was performed using the technique introduced by Kinmonth. For postoperative lymphography on the stumps of amputated extremities, two simple but useful methods were developed, which are presented here. Changes in the lymphographic appearance of lymph node metastases, the occurrence of new metastases, and the results of treatment were assessed by survey films and repeat lymphography. The generally accepted criteria for metastasis were used as a basis for the analysis of the lymphographic findings. The results may be summarized as follows: 1. Incidence of lymphatic dissemination. Different sarcomas varied greatly in their clinical course, including the frequency of dissemination. The lymphatic involvement in the metastatic cases was as follows: Bone sarcomas: 16 out of 28 (Table 10); of these, 13 were to regional lymph nodes, 8 to distant nodes and 5 to both (Table 14). Soft tissue sarcomas: 24 out of 40 (Table 11). All 24 had metastases in regional nodes, and 8 in distant nodes as well (Table 15). The highest frequencies of lymphatic spread in the different metastasized tumours were found to be: Bone sarcomas: reticulosarcoma 100%, Ewing's sarcoma 50%, osteosarcoma 47%. Soft tissue sarcomas: rhabdomyosarcoma 100%, synovial sarcoma 80%, neurogenic sarcoma 78%, leiomyosarcoma 67%. 2. Time-relation between lymphatic and haematogenic dissemination; The tendency to metastasize first via the lymphatics or via the blood vessels varied. Half of the cases of Ewing's sarcoma and reticulosarcoma had evidence of lymphatic spread before blood-borne metastases were detected. In the osteosarcoma cases, however, lymphatic dissemination was always preceded by haematogenic spread (Table 12). In synovial sarcoma, rhabdomyosarcoma and neurogenic sarcoma, the first dissemination was more frequently lymphatic than haematogenic (Table 13). 3. Possible existence of special lymphographic features of sarcoma metastases. Only reticulosarcoma displayed special characteristics. The lymph node metastases of reticulosarcoma of bone had lymphographic appearances similar to those found in reticulosarcoma of soft tissue or lymph node origin (Fig. 12). The lymph node metastases of other primary bone and soft tissue sarcomas had no specific lymphographic features and were indistinguishable from carcinomatous metastases (Figs 7, 9, 13, 15, 18, 19, 20, 22, 23). 4...

Adolescent↗

Fine needle aspiration cytology of sarcomas metastatic to the lung.

A review was made of the morphologic features of cells aspirated from 17 sarcomas (5 malignant fibrous histiocytomas, 3 fibrosarcomas, 3 leiomyosarcomas, 3 endometrial stromal sarcomas, 1 osteosarcoma and 2 poorly differentiated sarcomas) metastatic to the lung, paying particular attention to the morphologic differences between the cells of sarcoma and carcinoma and between the cells of the different types of sarcoma. In general, sarcomas were characterized by loosely cohesive, rather flat, cellular aggregates and isolated cells. Three-dimensional cell balls or clusters were not present in any case. Cellular pleomorphism was a common, though not invariable, feature. Each type of sarcoma showed some morphologic distinctiveness; however, certain morphologic features were common to more than one type of sarcoma. By comparing the cytologic features of metastatic sarcomas in aspirates with the histologic features of the primary neoplasms, it should usually be possible to decide if a pulmonary lesion is a metastatic sarcoma.

Biopsy, Needle↗

Primary tumours and tumour-like conditions of the mandible.

Reports on comprehensive mandibular tumours are very rare. The present article describes a total of 81 primary tumour and tumour-like lesions of the mandible seen at the University Hospital, Banaras Hindu University, during last 16 years. In the mandible the ratio of odontogenic to nonodontogenic tumors is 1.6:1. Ameloblastomas constitute 56% of the odontogenic tumors. Among the benign mesenchymal tumours of the jaw 58% are neoplastic and 42% are nonneoplastic in which cementoostoid tissue is laid down secondary to inflammatory process. Over all 40 cases are benign 28 potentially malignant (ameloblastoma) and 13 are malignant lesions. Among the malignant mandibular tumours, bone tumours are more common than the soft tissue sarcomas. Microscopically mandibular osteosarcomas are better differentiated than similar tumours in the long bones. Local, segmental, or radical surgery seems to be the treatment of choice in the mandibular tumours.

Adolescent↗

Alterations of blood analyses at relapse of osteosarcoma and Ewing's sarcoma.

Alterations of blood analyses have been studied at relapse of patients with osteosarcoma and Ewing's sarcoma. The tests included erythrocyte sedimentation rate (ESR), haemoglobin (Hb), leukocyte and thrombocyte counts, gamma glutamyltransferase (GT), lactate dehydrogenase (LD) and alkaline phosphatase (ALP). Sixteen relapsing patients diagnosed from 1970 to 1987 were eligible in each sarcoma group. Median age was 16 years (range 9-30) at diagnosis. The blood tests seemed to be of no help in detecting relapse of osteosarcoma, while ESR, LD and GT rose significantly in relapsing patients with Ewing's sarcoma. ESR was best correlated to disease activity.

Adolescent↗

Predictors of long-term survival with pulmonary metastasectomy for osteosarcomas and soft tissue sarcomas.

AIM: The control of pulmonary metastases is important for long-term survival of the patients with osteosarcomas and soft tissue sarcomas. METHODS: To evaluate the efficacy of aggressive pulmonary metastasectomy for those patients, we reviewed 105 cases (44 osteosarcomas, and 61 soft tissue sarcomas) treated in our hospital. RESULTS: The number of metastases was only the significant risk factor for the curability of the metastasectomy (logistic regression analysis, P=0.0274). The 5-year and 10-year survival rate were 43.6% and 32%. The curability was only independent prognostic factor on multivariate analysis (P=0.0008). CONCLUSIONS: Early detection of pulmonary metastasis, multimodal perioperative treatment, and design for minimizing the volume of resected lung tissues will enable complete and repeat resections for the achievement of long-term survival.

Adolescent↗