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Oncologic outcomes of primary bone sarcomas of the pelvis.

Although many studies have been performed for evaluation of surgical techniques and surgical morbidity in cases of primary pelvic sarcoma, few have addressed the long term oncologic outcomes for these patients. Therefore, the outcome of 41 patients with primary pelvic bone sarcomas were followed for 2-13 years (average, 6 years; median, 5 years). There were 18 chondrosarcomas (15 low grade and 3 high grade tumors), 11 osteosarcomas (1 low grade and 10 high grade tumors), 5 high grade malignant fibrous histiocytomas of bone, 5 Ewing's sarcomas, 1 undifferentiated sarcoma, and 1 low grade angiosarcoma of bone. Fifteen of the 17 patients with low grade tumors survived (88%), whereas only 6 of the 24 patients with high grade tumors survived (25%). Local disease recurred in 1 of the 24 (4%) patients with high grade tumors and in none of the patients with low grade tumors. Reoperations for complications were required in 33% of the patients. A difference could not be demonstrated in the quality of surgical margins or in the surgical morbidity, patient survival, and rate of local recurrence between patients treated with hemipelvectomy (8) and those treated with pelvic resection (32). Patients with low grade pelvic sarcomas have a good prognosis after pelvic resection, but those with high grade sarcomas continue to have a poor prognosis.

Adolescent↗

Ewing's bone sarcoma, paternal occupational exposure, and other factors.

To determine risk factors for Ewing's bone sarcoma, the authors interviewed mothers of 43 patients diagnosed between January 1978 and August 1986 and 193 controls in the San Francisco Bay Area, California, regarding medical and occupational history of parents and other factors related to the subjects and their immediate families. Controls were selected by using random digit dial telephone methods. Adjusted relative risk estimates suggest that risks were elevated for children whose fathers were engaged in agricultural occupations during the period from 6 months prior to conception of the subject up to the time of diagnosis for the patients or interview for the controls (relative risk (RR) = 8.8, 95% confidence interval (CI) 1.8-42.7) and for children whose fathers had occupational exposure to herbicides, pesticides, or fertilizers (RR = 6.1, 95% CI 1.7-21.9, p = 0.002). Prior ingestion of poison or an overdose of medication was more common in patients than in controls (RR = 4.4, 95% CI 1.4-13.5). These and other findings should be investigated in larger population-based studies to determine specific factors that may account for the associations.

Adolescent↗

[Current status of the treatment of high grade bone sarcomas].

The authors present a review of the changes occurred in the treatment of high grade bone sarcomas in the last 20 years. It was discussed the multidisciplinary approach for the local and systemic control of these tumors particularly the neoadjuvant chemotherapy that leads to an increase in the overall survival not only for the osteosarcoma but also for the Ewing sarcoma. They showed the possibilities for limb reconstruction after wide resection of the lesion that includes non conventional endoprosthesis and allografts.

Bone Neoplasms↗

Bone sarcomas.

The literature on radiotherapy for sarcomas originating in bone is limited, and the disease is uncommon. Consequently, this review is based on only 28 scientific articles, including 6 randomized studies, I prospective study, and II retrospective studies. These studies involve 1,394 patients. Radiotherapy is of limited value for treating the primary tumors of osteosarcoma. The effects of prophylactic lung irradiation for treating osteosarcoma are unconfirmed. Radiotherapy represents an integrated component of primary treatment for Ewing's sarcoma, but different fractionation schedules should be assessed. Radiotherapy is of limited value in the treatment of chondrosarcoma.

Bone Neoplasms↗

[Multipotent primary bone sarcoma].

The results of clinical and roentgen-morphological studies on 17 cases of multipotent primary sarcoma of the bone are presented. The difficulties of the tumor recognition are due to the fact that it occurs rarely, has no specific clinical-roentgenological picture and shows marked tissue polymorphism. Most frequent are differentiations of the type of osteogenic sarcoma, chondrosarcoma, reticulosarcoma with simultaneous combination with areas having the structure of Ewing's tumor, or extensive fields of undifferentiated primitive tumor cells. Further studies are necessary to decide whether or not this tumor is an independent form among other known skeletal neoplasias.

Adolescent↗

Endoprosthetic replacement following limb-sparing resection for bone sarcoma.

Prosthetic replacements are widely used in the reconstruction of deficits created by surgical resection for bone sarcomas. The longevity, complications, and functional outcome of these reconstructions vary by anatomic location, prosthesis type, and mode of fixation. Distal femoral replacement appears to be the most reproducibly successful prosthetic reconstruction, particularly when utilizing a cemented rotating-hinge device. Expandable prostheses may be the only alternative to rotationplasty or ablation in the young skeletally immature patient. Aseptic loosening and bone resorption are frequently noted complications of prosthetic replacement for which successful revision is nearly always possible. The incidence of infection has been reduced by use of better soft tissue coverage. The concept of extracortical bone bridging continues to evolve while enhanced tendon attachment emerges as a new development.

Bone Neoplasms↗

Evaluation of the effect of preoperative chemotherapy in bone sarcomas. 99mTc-HMDP scintigraphy in 34 cases.

We quantitatively analyzed images of 99mTc-HMDP bone scintigraphy in 34 bone sarcomas before and after preoperative chemotherapy. The isotopic uptake was calculated as tumor-to-background ratio (TBR), and the changes in TBR before and after chemotherapy were calculated as the alteration ratio. The histological effect of preoperative chemotherapy was classified in 4 grades, according to Huvos. We defined good scintigraphic responders as cases having decreased uptake after chemotherapy, with an alteration ratio > or = 60%. There was a positive correlation (Rho = 0.76, p < 0.001) between the histological effect and the alteration ratio. Of the 17 good histological responders, 13 showed a good scintigraphic response. The average alteration ratio in 17 cases with good response (68%) was higher than that in 17 cases showing poor response (-9.9%) (p < 0.001). 3 patients with pathologic fractures and 1 with primarily normal uptake showed false negative findings. The sensitivity of this method was 100% and the specificity was 81%.

Adolescent↗

[New approaches to antitumor therapy of various morphological variants of primary bone sarcoma].

The data of foreign literature and original findings on possible role of auto-, paracrine and endocrine mechanisms of bone sarcoma growth regulation are reviewed basing on observation in these tumors of receptors of the epidermal growth factor (REGF), EGF-like peptides, receptors of glucocorticoids, androgens and estrogens. Link of these indices with clinicomorphological characteristics of the disease and its prognosis is suggested. REGF in primary bone tumors may serve as targets for therapy with drugs influencing EGF-dependent mechanism of tumor growth stimulation. Groups of these drugs are considered in detail.

Antineoplastic Agents↗

Bone sarcomas in Paget disease: a study of 85 patients.

This is a comprehensive review of 85 patients who had bone sarcoma associated with Paget disease and who were seen at Memorial Sloan-Kettering Cancer Center between 1927 and 1982. There was an almost equal distribution of tumors in the axial and the appendicular skeletons. The pelvis, humerus, femur, and skull were the tumor sites in 80% of cases. The tumors were bulky large soft tissue masses. Lytic lesions were more common than sclerotic lesions. Mixed lytic and sclerotic lesions were much less common than either single type. Periosteal reaction was uncommon and found in less than 7%. Methylene diphosphonate scans of the bone often showed a cold area that was associated with marked increase in uptake on the gallium scan. Angiography, which was performed in 13 patients, was useful, but CT was much more helpful in showing the soft tissue mass as well as the extent of bony disease. Only three patients in this study survived for five years. Present chemotherapy protocols were disappointing in the treatment of this highly lethal tumor.

Adult↗

[The rehabilitative treatment of children with bone sarcomas].

The paper deals with the experience gained in carrying out rehabilitative surgery in 33 infant patients (37 operations) with bone sarcoma within 12 months to 12 years after combined treatment of primary tumor. Twenty-two operations using procedures of compression-distraction osteosynthesis were performed in 18 patients for post-surgery development of bone deformities and shorter limbs. shortening. Advantages of compression-distraction procedures are discussed since their application allows to make incisions and remove bone in areas outside the zone of radiation injury and permanent monitoring of exposed soft tissue condition. Twenty-two infants survived 5 years after orthopedic treatment. Four children died from metastases into the lungs 1-4 years after orthopedic rehabilitation.

Adolescent↗

Time and dose dependency of bone-sarcomas in patients injected with radium-224.

The time course and dose dependency of the incidence of bone-sarcomas among 900 German patients treated with high doses of radium-224 is analysed in terms of a proportional hazards model with a log-normal dependency of time to tumor and a linear-quadratic dose relation. The deduced dose dependency agrees well with a previous analysis in terms of a non-parametric proportional hazards model, and confirms the temporal distribution which has been used in the Radioepidemiological Tables of NIH. However, the linear-quadratic dose-response model gives a risk estimate for low doses which is somewhat less than half that obtained under the assumption of linearity.

Adult↗

Pulmonary metastases and bone sarcomas. Surgical removal of lesions appearing after adjuvant chemotherapy.

Pulmonary metastasis is the leading cause of death in pediatric patients with bone tumors. Multiple thoracotomies for surgical removal of individual lesions are performed at many centers. To explore the efficacy of this procedure and establish guidelines for an appropriate choice of patients, the experience with 43 thoracotomies in 24 children was reviewed. The appearance of first metastasis later than one year after diagnosis, presence of fewer than five nodules, and completeness of surgical resection were favorable predictors of postthoractomy survival. Early or multiple metastases, unresectable disease, and hilar, nodal, or pleural lesions conferred an unfavorable prognosis. With careful patient selection, pulmonary metastecotomy is a safe procedure that has few operative or long-term complications. It must be emphasized that although surgical removal of pulmonary metastases prolongs survival with good quality of life, the majority of patients with bone sarcomas ultimately succumb to their disease after thoracotomy. Follow-up time of greater than eight years is necessary to adequately assess the effectiveness of pulmonary resection in eradicating all disease in these patients.

Adolescent↗

Soft tissue and bone sarcomas in association with pregnancy.

The occurrence of cancer in a pregnant woman is a relatively infrequent event. The influence of pregnancy on the initiation, promotion and development of sarcomas is yet unclear. The medical approach is strongly influenced by the type and site of the primary tumor, its growth rate and associated symptoms, and by the need to treat the woman while minimizing fetal toxicity. The medical files of seven pregnant women in whom sarcomas were diagnosed during gestation or 3-4 months after delivery were reviewed. Three had bone sarcomas, and four had soft tissue sarcomas. In five cases there were various reasons for the delayed diagnosis and treatment, including lack of any signs or symptoms during gestation, clinical misdiagnosis, religious attitude and refusal of the patients. Ancillary tests such as magnetic resonance imaging (MRI) and ultrasound, and biopsy, are safe during gestation. Adriamycin, ifosfamide and granisetron were administered in one case during the third trimester, without remarkable toxicity. Whenever a pregnant woman is diagnosed as having a malignant tumor, serious medical, ethical, emotional, religious and philosophical dilemmas arise. The mother's well-being and the risk to the fetus by introducing early diagnostic tests and treatment should be weighed against the risk to the mother and the well-being of the fetus in carrying on an uninterrupted gestation.

Adult↗

Bone sarcomas in 239Pu-treated mice.

Female ICR mice were injected with a monomeric form of 239Pu (180.1 kBq/kg) at the age of 10 weeks and during the long-term contamination the incidence of bone sarcomas was studied. Average survival of 100 control mice was 545 +/- 14 days, the 100 plutonium-given mice survived 420 +/- 10 d and 50 sarcoma-bearing animals 456 +/- 11 days. Most of the 61 induced sarcomas were located in the spine (70%) and in trabecular parts of the other bones. Among these 61 tumors there were 55 osteoblastic osteosarcomas, 4 immature undifferentiated sarcomas and 2 angiosarcomas. In 6 tumor-bearing mice metastases were observed in lungs, liver, intracranial cavity and in mediastinal lymph nodes. Among control mice only one osteogenic mandibular sarcoma was verified. The osteosarcoma incidence was evaluated with respect to the retained activity and cumulative average skeletal dose. The results were discussed and compared with data published by others.

Animals↗

Prosthetic reconstruction of the femur for primary bone sarcoma.

The survival of patients and implants, complications and functional outcome were reviewed in 25 consecutive femoral endoprosthetic reconstructions for treatment of primary bone sarcoma. The diagnosis was chondrosarcoma in 11, osteosarcoma in 10, MFH in 3 and Ewing's tumour in 1. Median follow up was 64 months (34 to 219) and median age at operation was 29 years (10 to 70). Twelve remained disease free at review. One had amputation for local recurrence and another was alive with metastases. 11 patients died at a median of 13 months (5 to 128); 8 from metastatic disease and 3 from other causes. Four implants were revised, at a median of 95 months (53 to 136); two for fractures of the stem and two for aseptic loosening. Three implants had radiological evidence of loosening at a median of 43 months (34 to 49). Fourteen patients had significant complications. The median functional score using the Musculoskeletal Tumour Society system was 68%. In our experience, prosthetic reconstruction of the femur does not compromise survival, although there is a significant complication rate.

Adolescent↗