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Chondrosarcoma secondary to synovial chondromatosis. Report of two cases and a review of the literature.

Malignant transformation of synovial chondromatosis into chondrosarcoma is unusual. Thirteen cases and one series have been reported; only four of them developed in the hip. The overall survival is about 50%, possibly because of the difficulty of arriving at a correct early diagnosis (radiographically and histologically) and subsequent adequate surgical therapy. We report two patients (ages 30 and 50 years) in whom synovial chondrosarcoma developed in previously excised synovial chondromatosis of the hip. The diagnosis was made with modern imaging techniques (computed tomography and magnetic resonance imaging) and verified by open biopsy. The early recognition allowed a wide limb-saving resection; both patients are disease free 3 and 2 years after surgery.

Acetabulum↗

[Magnetic resonance tomography in osteoid osteoma: more confusion than benefit?].

The purpose of this evaluation was the description of potentially misleading MR appearances of osteoid osteoma. The MR images of 10 patients with osteoid osteoma were retrospectively evaluated and compared to radiographic, intraoperative and histologic findings. 4 of the 10 abnormalities were located in the proximal femur, two in the lumbar spine, and one each in the tibial plateau, in the cervical spine, in the sacrum and in the first metacarpal. 8 of the 9 nidi visible on standard radiographs and/or CT scans were demonstrated on MR images. Edema was visible within the bone marrow in 3, within soft tissue in 2 and in both locations in 4 MR examinations. The soft tissue abnormalities were circumscribed in 3 patients and could be misdiagnosed as soft tissue tumors or an abscess in these patients. One of 5 osteoid osteomas located in the proximity of a joint mimicked septic arthritis. The MR appearance of osteoid osteoma may be misleading. However, false diagnoses usually can be avoided with a careful search of a nidus. MR imaging in osteoid osteoma is important for differential diagnosis.

Adolescent↗

Sonographic diagnosis of bone tumors.

This paper presents the results of sonographic and radiographic examinations in 48 patients clinically diagnosed as having bone tumors. Sonography revealed bone destruction in all 48 cases, elevated periosteum in 26 cases and soft tissue mass in 34 cases. The results obtained in this series demonstrate that most bone tumors have their characteristic sonographic features such as giant cell tumors, malignant bone tumors, bone cysts, as well as metastatic lesions. Study showed that sonography has equally high accuracy in the diagnosis of these tumors compared with radiography.

Adolescent↗

Transepiphyseal extension of osteosarcoma: incidence, mechanism, and implications.

Forty-five cases of osteosarcoma were studied for transepiphyseal spread of the lesion because of the current interest in local resection and chemotherapy as treatment of this disease. In 17 cases, the epiphyseal plate had closed and all 17 had transepiphyseal extension of the tumor. In 28 cases, the epiphyseal plate was open; 21 showed growth or microscopic evidence of transepiphyseal extension of the lesion. The most common method of extension was directly through the epiphyseal plate along vascular channels, or less commonly, about the epiphyseal plate beneath the perichondrium and into the epiphysis along the epiphyeal vascular channels. The majority of these extensions were not detectable by either conventional radiography or radioisotope scanning. Local resection with preservation of the epiphysis will, in all likelihood, leave residual disease despite the oft-quoted statement that an open epiphyseal plate is a biologic barrier to the extension of bone tumors.

Adolescent↗

S-100 protein in tumors of cartilage and bone. An immunohistochemical study.

Using the peroxidase-antiperoxidase (PAP) immunohistochemical method, S-100 protein was found in well-differentiated chondrocytes of chondroma, chondroblastoma, mesenchymal chondrosarcoma, and osteosarcoma. It was not detected in osteoma, osteoblastoma, giant cell tumor, and Ewing's tumor. The presence of S-100 protein in tumorous chondrocytes and chondroblasts suggests that this protein may be a marker of chondrocyte origin and should not be considered a specific marker for nerve tissue.

Adolescent↗

Childhood multifocal osteosarcoma. Clinicopathologic and radiologic correlates.

The authors reviewed the clinical charts and pathologic and radiographic materials of nine pediatric patients (mean age, 11.0 years) presenting with multiple skeletal foci of osteosarcoma. Four patients presented with pulmonary metastases, and five had osseous disease only. All had densely sclerotic, predominantly osteoblastic tumors, usually in metaphyseal locations. Evidence of aggressive tumor cell behavior--such as vascular, articular, epiphyseal, or soft-tissue invasion--was usually present in surgical specimens. Despite intensive chemotherapy, all patients died from 6 to 37 months after diagnosis (median survival, 12 months). Comparison of clinical and pathologic features for patients presenting with or without pulmonary metastases failed to disclose any notable differences between the two groups, indicating a common pathogenesis and evolution for their multifocal disease. The authors conclude that this variant of osteosarcoma is a highly aggressive form of the disease that may be accompanied by pulmonary metastases.

Adolescent↗

Pediatric osteosarcoma. Successful retreatment of relapsed primary tumor and soft tissue recurrence with intraarterial cis-diamminedichloroplatin-II.

Six patients with extremity osteosarcoma initially underwent successful treatment of the primary tumor with four to seven courses of intraarterial cis-diamminedichloroplatin-II (IA-CDP; 100 to 150 mg/m2/course). The primary tumors in two patients were then extirpated by limb salvage. However, a local soft tissue recurrence occurred in both patients several months later. The other four patients refused surgical intervention and later also developed local bony recurrences. Reinstatement of three to five courses of IA-CDP in all six patients induced a complete response in five. The local recurrences (bony and soft tissue) in all six patients were excised. This included limb-salvage procedures in three patients. Additional postoperative chemotherapy was administered. Four patients remain alive and continuously free of disease 3.5+ to 4.5+ years after retreatment with IA-CDP.

Antineoplastic Combined Chemotherapy Protocols↗

Long-term survival after central nervous system relapse in a patient with osteosarcoma.

BACKGROUND: Since the advent of multiagent adjuvant chemotherapy, survival among patients with localized osteosarcoma has improved to 60% or more. Pulmonary relapse, the most common cause of treatment failure, is associated with less than 25% long-term survival; central nervous system (CNS) metastasis, when it occurs, often presents as a catastrophic clinical event in preterminal patients. METHODS: The authors report a patient with osteosarcoma who had an isolated pulmonary relapse and a subsequent isolated CNS relapse. Complete surgical resection was accomplished on both occasions and followed in the initial instance by adjuvant chemotherapy and in the latter instance by adjuvant whole-brain irradiation. RESULTS: An isolated CNS metastasis was resected and adjuvant whole brain irradiation was administered; the patient is alive and relapse free more than 5 years later. CONCLUSIONS: Patients with osteosarcoma in whom isolated, resectable metastases develop can derive benefit from gross total resection followed by adjuvant chemotherapy or radiation therapy for microscopic residual disease.

Brain Neoplasms↗

Comparison of two methods of reconstruction for primary malignant tumors at the knee: a sequential cohort study.

BACKGROUND AND OBJECTIVES: The purpose of this study was to compare the complications and functional outcome associated with the use of an irradiated allograft-implant composite or a bone-ingrowth modular tumor prosthesis for replacement of the knee joint after resection of a bone sarcoma from the distal femur or proximal tibia. METHODS: Eleven patients initially received an allograft reconstruction, followed by 64 treated with a tumor prosthesis. The primary analysis concerned reconstructive failure, defined by the requirement for removal of the original construct. Functional outcome was assessed by using the 1987 Musculoskeletal Tumor Society rating system. RESULTS: Reconstructive failure occurred in 6 of 11 (55%) allograft constructs compared with 10 of 64 (16%) tumor prostheses (P = 0.009). Failures were due to infection (2 of 11 allografts versus 4 of 64 prostheses; P = 0.2) or mechanical complications (4 of 11 allograft fractures versus 5 of 64 broken prosthetic stems and 1 aseptically loose prosthesis; P = 0.03). The limb salvage rate was 95% (61 of 64) for patients with a tumor prosthesis compared with 64% (7 of 11) for those with an allograft (P = 0.007). Patients with a tumor prosthesis had a better functional outcome with a mean score of 75% compared with 57% for those with an allograft reconstruction (P = 0.006). CONCLUSIONS: This comparative study suggests that limb salvage surgery at the knee has a better and more predictable outcome with a tumor prosthesis than with an allograft-implant reconstruction.

Adolescent↗

Limb salvage surgery for locally aggressive and malignant bone tumors.

Since 1983, 77 patients have had limb salvage surgery for locally aggressive or malignant bone tumor of the limbs or pelvis. Twenty-nine patients had locally aggressive lesions with giant cell tumors the most prevalent, and 48 patients had malignant lesions, of which osteosarcomas predominated. Resection with a wide margin could be achieved in most of the patients. A mobile joint reconstruction was performed in 57 patients and an arthrodesis in 14 patients. At follow-up, 14 patients have died of disease, 7 patients have survived with disease, and 56 patients (73%) have survived without disease. Local recurrences were encountered in 4 patients (5%). The overall functional results were classified as excellent in 11 patients, good in 45 patients, and fair in 9 patients. Twelve patients had poor results because of failure of the reconstruction or amputation due to complications. Twenty-seven complications were encountered, among which infection was the most serious. Careful selection of patients and consideration of procedure chosen to reconstruct the defect are important for a successful outcome. This decision is based on a number of factors related to the tumor and the patient. Presently, major neurovascular involvement, displaced pathological fracture, inappropriate biopsy incision, extreme youth of the patient, and infection were considered to be contraindications to resection in our institution. Although various procedures promise functional restoration, the reconstructive procedure should be individualized and designed to meet the needs of the patient.

Adult↗

Vascularized fibular graft for bone reconstruction of the extremities after tumor resection in limb-saving procedures.

We treated 18 patients (8 males and 10 females) with bone or soft tissue tumors in the extremities by vascularized fibular grafts (VFGs). The average age was 29 years. Two had malignant soft tissue tumors in the forearm (angiosarcoma and synovial sarcoma) and the other 16 had bone tumors [osteosarcoma (8), chondrosarcoma (3), angiosarcoma (1), adamantinoma (1), and giant cell tumor (3)]. Affected sites of the bone tumor cases were tibia (5), femur (5), humerus (3), radius (2), and ulna (1). According to the surgical staging system of Enneking et al. 1 patient was in stage III, 12 in IIB, 2 in IB, and 3 in IA. The surgical margin was curative in 5 patients, wide in 9, marginal in 2, and intralesional in 2. One patient died due to lung metastasis although bone union was obtained by the VFGs. Functional results of the bone reconstruction in the remaining 17 patients were evaluated according to the modified scale of Enneking et al. The VFGs resulted in substantial bone unions in all patients except 1. Bone unions in all 17 patients occurred in less than 10 months. Overall clinical results were satisfactory. Based on a 30-point scale overall clinical results ranged from 10 to 30 points with an average of 21 points. Functional evaluation in the upper extremity were, generally, superior to those in the lower extremity. Postoperative complications were 5 metastases (4 in the lung and 1 in the mediastinum) in 4 patients, 1 recurrence, and 2 fractures after obtaining bone union. One patient died from metastasis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Osteogenic sarcoma following treatment with megavoltage radiation and chemotherapy for bone tumors in children.

While osteogenic sarcoma has been well-recognized as a late complication of exposure to high doses of ionizing radiation in the orthovoltage energy range, it has been less frequently reported in patients treated with megavoltage radiation. This potential complication should, however, not be dismissed as an occurrence to be seen only after high-dose orthovoltage radiation. We have recently seen two children who developed osteogenic sarcoma following treatment with megavoltage radiation and combination chemotherapy for primary bone tumors. The implications in regard to aggressive multimodality treatment for pediatric malignancies are discussed.

Adolescent↗

Two freezing cycles ensure interface sterilization by cryosurgery during bone tumor resection.

Cryosurgery utilizing an argon-based system allows bone-tumor interface sterilization, while avoiding the risks of conventional cryosurgery. This study was conducted in order to evaluate the number of freezing cycles required for interface sterilization in cases of aggressive human bone tumors. Sixteen tumors were included (six chondrosarcomas, eight metastatic carcinomas, and two giant cell tumors). All occurred within long bones. In all cases a standardized marginal resection was performed. Following thorough curettage, we sampled five different locations within the tumor interface by a cylindrical hollow trephine. The interface viability was assessed using the XTT method. Quantitative histological evaluation was based on the percentage of live cells divided by total lacunae number in five random medium-power fields. One freezing cycle (5 min, -40 degrees C) reduced tumor viability to approximately 5% of prefreezing. However, there were still live specimens. Two or three freezing cycles led to complete interface sterilization. The difference between a single freezing cycle and two freezing cycles was significant (ANOVA, F = 130, P < 0.01). The difference between two freezing cycles and three freezing cycles was not significant (ANOVA, F = 0.14, P < 0.6). The results of the XTT method for the assessment of interface viability correlated well with histological evaluation of the percentage of viable cells (r = 0.89), as well as with cell culture results of frozen vs. prefreezing tumor samples. In conclusion, two freezing cycles are adequate to achieve tumor-bone interface sterilization in aggressive human bone tumors.

Adult↗

Local growth and the prognosis of osteosarcoma.

We have analysed 63 patients who had stage II osteosarcoma of the distal femur (32) or proximal tibia (31) to determine the patterns of disease-free and overall survival in relation to local growth. All had neoadjuvant chemotherapy. The extent of local growth predicts both types of survival in these tumours.

Adolescent↗

Hemijoint allografts in the treatment of low grade malignant and aggressive bone tumours about the knee.

Nine consecutive patients undergoing osteoarticular allogenic transplantation for low grade malignant or aggressively benign tumours have been followed up for between 8 to 15 years. Eight patients had functioning knees whilst 1 ended in arthrodesis. Of the functioning knees all had full extension but only 3 could flex more than 90 degrees. Seven patients had returned to work. Radiologically all the knees showed progressive deterioration. In spite of this only three knees were painful with exercise and none at rest. We conclude that hemijoint allografts represent a useful alternative to make good a bony defect after tumour resection about the knee, but that the ultimate result of such a reconstruction depends on survival of the joint surfaces.

Adolescent↗