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Giant-cell tumour of bone metastasising to the lungs. A long-term follow-up.

We reviewed 23 patients with a giant-cell tumour (GCT) of bone and histologically-proven lung metastases at a mean of 11.9 years (3 to 24.5) after the original diagnosis. The mean age of the patients at diagnosis was 27.3 years (9 to 61); the male to female ratio was 0.9:1. The most common primary site was the distal radius. The mean interval between the onset of the tumour and the detection of lung metastases was 4.1 years (0 to 24). There had been local recurrence in 19 (83%) either before or at the time of diagnosis of lung metastasis. Surgical resection alone was the preferred treatment for lung metastases in 70% and resulted in 18 patients (76%) being free from disease when last reviewed. One patient had the spontaneous regression of lung metastases and was free from recurrence 16 years later. Sixteen patients (69.7%) have survived and four (17.4%) have died from progression of the tumour. Three other deaths were not related to the tumour. Local recurrence and a primary lesion at the distal radius seem to be associated with an increased risk of lung metastases. Repeated surgical resection of lung metastases gave a high rate of survival.

Adolescent↗

Osteoid osteoma. Direct visual identification and intralesional excision of the nidus with minimal removal of bone.

We describe 100 consecutive patients with osteoid osteoma. Of the 97 who had operations, 89 were treated by intralesional excision and eight by wide resection. The three remaining patients were not operated on because the osteoid osteoma was almost painless, or was found in the pedicle of the 12th thoracic vertebra at the site of entrance of the artery of Adamkjewicz. The diagnosis was confirmed histologically in all specimens. No local recurrences were observed at a minimum follow-up of one year. All except one patient were mobilised two to four days after surgery. A precise preoperative diagnosis of the lesion is mandatory, based on clinical findings, standard radiographs, thin-section CT and a bone scan. We compared our operative technique with 247 cases in which the percutaneous technique of removal or coagulation of the nidus had been performed. The latter procedure has a less constant rate of primary cure (83% v 100%). Its principal indication appears to be for osteoid osteomas in the proximal femur and the pelvis.

Adolescent↗

Intercalary endoprosthetic reconstruction for diaphyseal bone tumours.

Custom-made intercalary endoprostheses may be used for the reconstruction of diaphyseal defects following the resection of bone tumours. The aim of this study was to determine the survival of intercalary endoprostheses with a lap joint design, and to evaluate the clinical results, complications and functional outcome. We retrospectively reviewed six consecutive patients, three of whom underwent limb salvage with intercalary endoprostheses of the tibia, two of the femur, and one of the humerus. Their mean age was 42 years (28 to 64). The mean follow-up was 21.6 months (9 to 58). The humeral prosthesis required revision at 14 months owing to aseptic loosening. There were no implant-related failures. Musculoskeletal Tumour Society functional outcome scores indicated that patients achieved 90% of premorbid function. Custom intercalary endoprostheses result in reconstructions comparable with, if not better than, those of allografts. Using this design of implant reduces the incidence of early complications and difficulties experienced with previous versions.

Adult↗

Survivorship and clinical outcome of modular endoprosthetic reconstruction for neoplastic disease of the lower limb.

We reviewed retrospectively the results in 211 consecutive patients who had undergone limb salvage for bone neoplasia with endoprosthetic reconstruction of the proximal femur (96), distal femur (78), proximal tibia (30) and total femur (7). Their mean age was 50 years (11 to 86) and the mean follow-up period was 37.3 months (1 to 204). A total of 35 (16.6%) prostheses failed. Overall, implant survival was 78% (95% confidence interval (CI) 0.29 to 0.54) at five years, 60% (95% CI 0.93 to 2.35) at ten years and 60% (95% CI 1.27 to 3.88) at 15 years. Survivorship of the limb was 97.6% (95% CI 1.73 to 3.35) at ten years. The gender, age, diagnosis and location of the tumour were not prognostic variables for failure. Modular endoprosthetic replacement in the lower limb is a durable long-term reconstructive option, with the implants generally outlasting the patient.

Adolescent↗

Prosthetic survival and clinical results with use of large-segment replacements in the treatment of high-grade bone sarcomas.

We evaluated the long-term clinical results and the survival of the prostheses in eighty-two patients who had had a limb-sparing procedure by means of the implantation of a large-segment prosthesis. All patients had had a high-grade bone sarcoma of the distal, middle, or proximal part of the femur; the proximal part of the humerus; the proximal part of the tibia; or the pelvis. The duration of follow-up ranged from two to twelve years (median, three and one-half years). Function was evaluated with the revised 30-point classification system of the Musculoskeletal Tumor Society. The survival of the prostheses was analyzed with regard to several variables with use of Kaplan-Meier survival estimates. Sixty-eight patients were alive at the latest follow-up evaluation. The survival rate of the prostheses was 83 per cent at five years and 67 per cent at ten years. Twelve prostheses were revised, and eleven revisions were successful. The rate of revision was highest (six of thirteen) in the patients who had had a tumor of the proximal part of the tibia. In contrast, only three (10 per cent) of the thirty-one patients who had had a tumor of the distal part of the femur and three (10 per cent) of the twenty-nine who had had a tumor of the proximal part of the humerus had a revision. Eleven patients (13 per cent) had an infection, which necessitated an amputation in six. Five patients (6 per cent) had a local recurrence, and nine patients (11 per cent), including the six already mentioned, ultimately needed an amputation. Patients who had had a tumor of the proximal part of the humerus had the highest functional scores, while those who had had a tumor of the proximal part of the tibia had the lowest scores. Large-segment prostheses were a good reconstructive option for the treatment of high-grade bone sarcomas in our patients. The rates of long-term survival of the prostheses were acceptable and the functional results were good or excellent after this form of treatment at most of the anatomical sites at which they were used.

Adult↗

Malignant tumor of the distal part of the femur or the proximal part of the tibia: endoprosthetic replacement or rotationplasty. Functional outcome and quality-of-life measurements.

BACKGROUND: The present study was performed to determine whether there is a difference, with regard to functional outcome and quality of life, between endoprosthetic replacement and rotationplasty for the treatment of malignant tumors of the distal part of the femur or the proximal part of the tibia. METHODS: Sixty-seven patients, between the ages of eleven and twenty-four years at the time of the diagnosis, had a malignant tumor of the distal part of the femur or the proximal part of the tibia. A rotationplasty was performed in thirty-three patients, and an endoprosthetic replacement was done in thirty-four patients. The median duration of follow-up was six years and one month (range, two years to sixteen years and two months). The scale developed by the Musculoskeletal Tumor Society was used to evaluate the functional results. Quality-of-life issues were assessed with the questionnaire developed by the European Organization for Research and Treatment of Cancer. RESULTS: The patients who had had a rotationplasty had a mean functional score, according to the system of the Musculoskeletal Tumor Society, of 24 points, and the patients who had had an endoprosthetic replacement had a mean score of 25 points. This difference was not found to be significant, with the numbers available (p = 0.47). Only one patient who had had a rotationplasty used an assistive device when walking long distances, whereas six patients who had had an endoprosthetic replacement used an assistive device. This difference was significant (p<0.001). The quality-of-life questionnaire revealed that the patients who had had a rotationplasty could participate in hobbies such as carpentry and sports as well as in other daily activities to a significantly greater degree than those who had had an endoprosthetic replacement (p = 0.001). Restriction in daily activities due to pain was significantly less common in the group that had had a rotationplasty than it was in the group that had had an endoprosthetic replacement (p = 0.047). CONCLUSIONS: Rotationplasty was not associated with any disadvantages with regard to function or quality of life in comparison with endoprosthetic replacement. It is possible that the psychosocial outcome is influenced by the fact that patients who have a rotationplasty know that additional operative intervention is not usually necessary. Despite good functional and quality-of-life results, the cosmetic appearance may be the most serious disadvantage of rotationplasty. The decision to perform this procedure must be made on a case-by-case basis.

Adolescent↗

PET/CT characterization of fibroosseous defects in children: 18F-FDG uptake can mimic metastatic disease.

OBJECTIVE: The purpose of this study was to characterize the anatomic appearance and metabolic activity of nonossifying fibromas, fibrous cortical defects, and cortical desmoids on PET/CT images. CONCLUSION: Over a 14-month period, we identified eight nonossifying fibromas, four fibrous cortical defects, and two cortical desmoids in 330 children who underwent PET/CT for the evaluation of a known or suspected malignancy. CT, conventional radiography, MRI, or clinical follow-up was used to confirm the diagnoses of these fibroosseous lesions. Eleven of the 14 children underwent multiple PET/CT examinations; thus, 34 studies were included. The lesions showed variable metabolic activity as indicated by 18F-FDG uptake: 19 PET/CT examinations showed lesions with mild 18F-FDG uptake, eight showed moderate 18F-FDG uptake, and seven showed intense uptake. When PET reveals metabolically active osseous abnormalities in children who are at risk for bone metastases, benign fibroosseous lesions should be considered in the differential diagnosis before additional diagnostic procedures are undertaken. Benign fibroosseous lesions may be metabolically active and thus mimic metastatic osseous disease in children with underlying malignancies. Correlative CT or other anatomic imaging can confirm the benign nature of these lesions.

Adolescent↗

Symptomatic bursa formation with osteochondromas.

Two cases of bursa formation in association with osteochondromas are presented. This condition may be confused radiographically and clinically with malignant transformation of the cartilage cap. Ultrasound examination on one of the patients proved helpful in arriving at the correct preoperative diagnosis. Ultrasonography was also helpful to the surgeon with regard to size and extent of the bursa.

Adult↗

Treated Ewing sarcoma: radiographic appearance in response, recurrence, and new primaries.

Healing of Ewing sarcoma after therapy has a predictable radiographic appearance, eventually stabilizing and remaining constant in appearance. This study is based on 35 patients in whom adequate follow-up radiographs were available. The expected sequence was evident in 21 patients. Complete healing was evident in about 1 year. Failure to heal as expected or lytic change in healed bone indicated recurrent or refractory disease in 11 patients who developed distant metastases or clinical signs of recrudescence at the primary site. This complication was evident within months to 2 years of the initial treatment. In three patients, development of a new, primary, osteogenic sarcoma was heralded by disorganized tumor new bone in an osteoid matrix, evident only after several years of stability. These new osseous malignancies are probably treatment-related.

Adolescent↗

Specimen radiography of bone tumours.

Radiographs obtained with a transportable roentgen apparatus and a shielded cabinet on fine grain films of whole specimens and/or slabs of transsected bone in primary bone tumours and soft tissue tumours with skeletal invasion or ossification were compared with clinical roentgenograms and related to gross and microscopic pathology as demonstrated in selected cases. Specimen radiography is found to be a valuable adjunct to standard morphologic examination and forms a link to clinical radiology in the study of bone tumours and related diseases.

Adolescent↗

Epiphyseal osteoid osteoma. Two case reports.

Two cases of epiphyseal osteoid osteoma presented with a 1-2 year history of monarthritis with swelling and impaired function of the knee and ankle, respectively. In both cases, removal of the lesion resulted in complete recovery.

Adolescent↗

Parosteal osteosarcoma. 2-23-year follow-up of 33 patients.

In a study of the working group for bone tumors of the German Orthopedic Society, 33 patients with a histologically confirmed parosteal osteosarcoma at reexamination underwent clinical and radiographic follow-up. Local recurrence occurred in all the cases after intralesional surgery and in 4 of 8 cases after marginal excision. The grade of differentiation was decisive for the prognosis. Despite intralesional surgery, the prognosis for Grade 1 tumors was good. Metastases developed in 1 of 23 patients with a Grade 1 tumor and in 4 of 9 patients with a Grade 2 tumor. The single patient with a Grade 3 tumor was treated with adjuvant chemotherapy and was free of disease after 5 years.

Adolescent↗

[Detection of p53 and MDM2 gene expression in osteosarcoma with biotin-labelled in situ].

We studied the mRNA expression of p53 and MDM2 gene in osteosarcoma. Twenty osteosarcomas were examined with biotin-labelled oligodeoxynucleotide probe in situ. Of 20 tumors, 8 (40%) and 6(30%) showed positive expression of p53 and MDM2 genes, respectively. Among these, 2 tumors revealed co-expression of both p53 and MDM2 genes. This study confirmed the existence of aberrant mRNA expression of p53 and MDM2 genes in osteosarcoma.

Adolescent↗

Osteoid osteoma. Computed tomography guided percutaneous excision.

The authors' experience with computed tomography guided excision of osteoid osteoma in an outpatient setting using an Einhorn biopsy set and a power drill in 21 patients is described. In the last 5 years, 21 patients aged 7 to 51 years who had radiographic findings and symptoms of osteoid osteoma were included in the study. The nidus of the tumor was excised successfully in all cases using a coaxial technique with a power drill. Excision of the nidus was confirmed by postexcision computed tomography scans, histologic evaluation, and absence of symptoms on clinical and radiographic followup. Only one minor complication and no recurrences occurred with a mean followup of 17 months (range, 9-43 months). Percutaneous computed tomography guided excision is an effective, safe, and cost effective method of treatment of osteoid osteoma.

Adolescent↗

[Bone localizations of Hodgkin's disease].

Bone lesions have been observed in 43 out of 415 Hodgkin's disease patients (9.6%): the radiographic findings are discussed with reference to clinical problems.

Adolescent↗