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Brain metastases in osteosarcoma. Report of a long-term survivor and review of the St. Jude Children's Research Hospital experience.

BACKGROUND: Brain metastasis has been considered a rare event in osteosarcoma, although with prolonged survival an increasing incidence has been suggested. There have been no prior reports of long-term survivors among patients with this complication. METHODS: The authors describe a child treated for osteosarcoma who is alive and free of disease 8 years after the detection of brain metastases. Of 254 patients with primary osteosarcoma referred to St. Jude Children's Hospital between 1962 and 1989, 13 developed brain metastases, all after relapse or recurrence in another site. Concomitant active lung metastases were present in all of the patients except the one long-term survivor, whose pulmonary disease had responded to treatment with cisplatin and doxorubicin. Log-rank analyses were used to compare survival duration and the frequency of brain metastases among patients treated before and after 1982, when effective multiagent therapy was initiated. RESULTS: Log-rank analyses comparing patients treated before and after 1982 showed that the introduction of effective modern therapy improved survival among patients at risk for brain metastases (i.e., those with recurrent and progressive disease, P = 0.007) but was not associated with a statistically significant increase in the frequency of brain metastases (15.5% versus 4.5%, P = 0.125). CONCLUSIONS: Although the outlook for patients with this complication remains bleak, the resolution of brain metastases after eight courses of ifosfamide in the patient described in this article suggests that enrollment of selected patients in Phase II trials is merited.

Adolescent↗

Structural consequences of endosteal metastatic lesions in long bones.

Lytic metastatic lesions from breast, prostate, and other cancers often develop on the endosteal surface of a long bone without penetrating the cortical wall. Current clinical guidelines for determining the fracture risk associated with these endosteal defects do not account for the structural consequences of the lesion. We undertook a combined experimental and analytical study of the structural consequences of the lesions with the ultimate goal of providing improved fracture risk guidelines. Endosteal defects of variable length and involving a variable amount of the cortical wall were created with an expanding reamer in canine femurs. The contralateral femur served as a control. The femurs were tested to failure in four point bending. The geometry of the experimental defects was determined from radiographs and CT. Finite element models of the canine femurs were then used to examine geometric and material parameters in both four point bending and in torsion. The experimental data demonstrate a linear relation between bone strength and amount of cortical wall remaining: % intact strength = 99.6 x remaining wall thickness - 2.0, R2 = 0.769, standard deviation of regression = 11.57. Four of five data points from the linear finite element models were within the 95% confidence intervals for the experimental data. Experimental and finite element data suggest that the minimum wall thickness is the most critical geometric parameter for predicting the structural consequences of endosteal defects. The length of the defect along the bones' long axis has little effect on bone strength. The anelastic behavior of bone does not need to be represented in finite element models of simple endosteal defects because the defects do not cause significant stress concentrations. However, reduction in the modulus of bone along the border of a defect (due to osteolytic changes) can significantly reduce bone strength. These results indicate that the minimum wall thickness should be determined when clinically evaluating an endosteal defect. The results also suggest that information on bone porosity around metastatic lesions should be considered when making estimates of bone strength.

Animals↗

Free vascularized fibula in traumatic long bone defects and in limb salvaging following tumor resection: comparative study.

In this retrospective analysis, we present our experience with two groups of patients who had long bone defects secondary to trauma or tumor resection and who were treated with a free vascularized fibular graft for skeletal reconstruction. Both groups were comparable in number and average age of patients, length of bone defect, and mean follow-up (average 3 years both groups). The number of surgical procedures prior to microvascular grafting was significantly higher for the traumatic defects. Primary bone union in a mean period of 6 months occurred at a higher rate in the tumor patients; the trauma patients had a significantly higher nonunion rate, which required multiple additional surgical procedures. The latter did not, significantly, improve the rate of success in the trauma group. Residual limb shortening was present in one-half of the patients with traumatic defects. On the basis of this review, it appears that the scarred and relatively avascular soft tissues surrounding the long bone defects secondary to trauma affect the course and the final outcome of the microvascular fibular grafting. A similar procedure applied for limb salvaging after tumor resection is better.

Adolescent↗

Breast cancer after treatment for osteosarcoma.

Two cases of patients with primary osteosarcoma who developed subsequent new primary infiltrating ductal carcinoma of breast are presented. The relationship of irradiation from diagnostic radiology, chemotherapy given, and possible genetic factors are discussed. A recommendation for the lifetime follow-up program of a patient with osteosarcoma should include careful attention to breast self-examination and regular breast examination by the attending physician.

Adenocarcinoma↗

Isolated CNS involvement in Ewing's sarcoma.

Ewing's sarcoma, an uncommon malignant neoplasm of bone, represents about 10% of all the malignant primary bone tumors. The assumption that subclinical metastases are already present in patients with apparently localized tumor indicates the need for systemic therapy as an integral part of primary treatment. The usual sites of metastases are the lungs and skeletal system. Central nervous system (CNS) involvement is rare and is usually seen only in disseminated and fairly advanced disease. We report two patients in whom, after aggressive adjuvant chemotherapy, disease developed in the CNS without local recurrence. In one patient, meningeal involvement with malignant cells was identifiable in the cerebrospinal fluid; in the second patient, who presented with a space-occupying lesion, a diagnosis of arachnoid involvement was made histologically.

Adolescent↗

Incidence of regional lymph node metastasis in operable osteogenic sarcoma.

Records of 182 consecutive patients with the diagnosis of operable osteogenic sarcoma, treated between 1954 through 1980 by the Breast, Bone and Mixed Tumors Department of the Instituto Nacional de Enfermedades Neoplasicas (Lima, Peru), were reviewed to study the incidence of regional lymph node metastases in this disease. All the patients included in this study had radical surgery, which means the complete resection of the bone where the tumor is located, including the proximal joint, which permitted excision of the regional lymph nodes. Nineteen patients (10.4%) had evidence of sarcoma metastatic to draining lymph nodes. A comprehensive analysis of the literature shows that the incidence of metastasis to the lymph nodes in this study, is higher than those cited in a review of the literature, probably due to the policy in the management of osteogenic sarcoma in our institution, during the period of study.

Adolescent↗

Metastatic fractures of long bones.

Forty-two patients with a total of 52 metastatic fractures of long bones were analysed retrospectively with regard to the type of treatment, complications, general mobility and survival. Endoprosthetic replacement appeared to be better than osteosynthesis in the proximal femur, and this method should also be considered in pathological fractures of the distal femur. Nailing is indicated in fractures of the shafts of long bones in the lower limb, and also in the humerus. Osteosynthesis with a plate has only limited indications. Cement should be added to an osteosynthesis or replacement of a pathological fracture in order to achieve immediate stability. On the whole, the life expectancy of these patients has improved; five in our series lived for more than 6 years after the treatment of their metastases. The method of surgical treatment therefore needs more consideration.

Bone Neoplasms↗

The use of corundum ceramic implants after excision of tumours of the extremities.

Implants of corundum ceramic have been used in the treatment of 38 patients with bony tumours of the limbs. Data are presented concerning the replacement with ceramic implants of the joint ends and diaphyseal defects of long bones, as well as in various bones of the hand and foot. Experiments carried out over a long period have shown that corundum ceramic is a strong material and is biologically inert.

Adolescent↗

Temporary resection-arthrodesis of the knee using an intramedullary rod and bone cement.

A temporary arthrodesis of the knee, using an intramedullary Küntscher rod and bone cement, was carried out in 76 patients after resection of tumours of the distal femur or proximal tibia. In contrast to primary arthrodesis with bone grafting, this procedure allows the patient to bear weight on a stable limb a few days after operation, reduces the risk of infection and avoids the negative effect of chemotherapy on graft incorporation. In 12 cases (16%) the implant failed mechanically by fracture, or by bending or migration of the rod, at from 13 to 54 months after operation. Deep infection occurred in 11 patients (14%). A second reconstruction was carried out in 14 patients, 10 using Kotz prostheses and 4 arthrodeses with bone grafting. The operation we describe has advantages for patients with high grade tumours which require chemotherapy and resection-arthrodesis of the knee joint.

Adolescent↗

[Experimental methods of creating V X 2 bone tumour in rabbits (author's transl)].

V X 2-carcinomas were induced in rabbit femurs by various methods. Inoculation near the periosteum after injury of the condylus medialis femoris was considered most successful. Results of 124 inoculations and 10 controls are demonstrated by angiograms, pharmakoangiograms and histologic photographs. Injury of periosteum and bone favors the taking and spreading of malignant growth.

Angiography↗

[Atypical amputations of bone tumors of the lower extremity (author's transl)].

The most predominant problem concerning malignant tumors in the lower extremities is the radical removal of the tumor, for this almost always involves amputation. Referring to the eventual outfitting of a prosthesis one must keep functionality in mind from the start. This includes all the planing for the preservation of a stump that can be fitted with a prosthesis. Experience has confirmed the advantages of foot-root stumps and knee disarticulation. Good functional results can be attained with tumors of the femur in individual cases through atypical operations. This involved elongation of the stump by means of a fibula transplant; replacing the femur with a bioceramic endoprosthesis; and rotation-plasty, whereby the knee joint is replaced by the ankle joint.

Adolescent↗

Is bone grafting necessary? Analysis of twenty cases of giant cell tumour of bone treated by curettage without graft.

Twenty cases of giant-cell tumour of bone treated over an 11-year period were analysed. The minimum follow-up was 2 years. Primary treatment consisted of a detailed curettage alone, without bone grafting. A recurrence rate of 30% (six patients) was recorded. Cases of recurrence were suitable for resection and endoprosthetic replacement. We conclude that detailed curettage alone, without a bone graft, is an effective primary treatment for patients with giant-cell tumour of bone.

Adolescent↗

[Results of treatment of tumors and metastases of the pelvis and proximal femur].

A retrospective study of 293 primary tumors and 244 metastases of the pelvis and proximal femur allows the following conclusions. Benign tumors are treated by curettage whereas low-grade malignant tumors have to be resected "en bloc". In high-grade malignant pelvis tumors, total internal hemipelvectomy with replacement of half of the pelvis has been used with success. Metastases are treated by total hip prosthesis or a combination of bone cement and osteosynthesis. On the whole early lethality is low, local complications are acceptable and most of the patients are able to walk.

Adolescent↗

Magnetic resonance imaging of intraosseous lipomas: a radiologic-pathologic correlation.

Four patients with intraosseous lipomas were studied with magnetic resonance imaging. The imaging features and histology of each tumor were compared. Magnetic resonance imaging was very helpful in establishing a pathologic diagnosis. If a severe degree of involution was present, then the magnetic resonance findings could be ambiguous, making diagnosis more difficult.

Adipose Tissue↗

[Bone metastases of breast cancer].

Bone metastasis in hip region of a mammary cancer are not that often. This case should show the problems growing with that kind of tumorous way.

Adenocarcinoma, Scirrhous↗

[Amputation or limb salvage? Assessing quality of life after tumor operations of the lower extremity].

The purpose of this study was to compare quality of life and subjective well-being between patients who underwent either amputation or limb-salvage procedures for lower extremity sarcomas in order to determine if this aspect can be considered in the decision on the type of surgery. Sixty-six patients were evaluated at least 1 year after surgery and systemic therapy for lower extremity sarcomas. Self-report questionnaires such as the Quality of Life Questionnaire (QLQ-C30), the Life Satisfaction Questionnaire (FLZ), and the Enneking Score (MSTS) were used. There were no differences in quality of life and subjective well-being between the two groups. Scores of the symptom scale (14.1 vs 13.2), the global quality of life (70.9 vs 71.0) as well as scores of the function scale (51.7 vs 50.3) were similar. Small differences in scores were found in social functioning but not in scores of physical, cognitive, and emotional functioning. Global subjective well-being in both groups was also similar (219.5 vs 223.7). High satisfaction was found in marital life and partnership as well as in self-assessment. The least satisfaction was found in finances. Results in functional outcome after limb-salvage procedures (77% vs 65%) were better. Although more complications were reported after limb-salvage procedures, they did not seem to have any impact on quality of life ( p=0.235, r(p)=0.112) and subjective well-being ( p=0.856, r(p)=0.140) of the patients whereas a correlation to function could be shown ( p<0.001, r(p)=0.595; p=0.015, r(p)=0.304). Additionally, a correlation between subjective well-being and the location of either amputation or reconstruction ( p=0.039, r(p)=- 0.309) could be shown. These findings demonstrate that the type of surgery has no influence on quality of life and subjective well-being after lower extremity sarcomas. Thus the decision for either limb-salvage procedures or amputation cannot be based on quality of life and subjective well-being but must be determined by oncological criteria.

Activities of Daily Living↗