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[Rotationplasty in the surgical treatment plan of primary malignant bone tumors. Possibilities and limits].

Long-term survival of patients with a malignant bone tumor increased with a combined therapeutic approach using chemotherapy and possibly radiation therapy. Today up to 80% of patients with an osteosarcoma or Ewing's sarcoma survive. Therefore, it is important that the functional outcome and the quality of life are good after resection of the tumor. Before the era of endoprosthetic devices, rotationplasty, which was introduced in 1974 by Salzer for surgery of malignant bone tumors and modified by Winkelmann, protected many patients from an amputation. Although many authors favor limb-saving procedures today, rotationplasty obtained excellent functional and psychosocial results. Rotationplasty can be recommended in tumors with a large soft tissue component, in tumors located in the proximal femur and proximal tibia, and in children under 10 years of age. Furthermore, rotationplasty can be used in patients in whom a limb-salvage procedure failed because of infection or repeated changes of the endoprosthesis.

Adolescent↗

[Endoprosthesis management of the extremities of children after resection of primary malignant bone tumors].

In recent years, significant progress has been made in the treatment of primary malignant bone tumors. The surgeon is confronted with increasing demand for limb-sparing surgery by patients and parents alike. In the skeletally immature, resection of the growing physis will ultimately result in leg length discrepancies and functional impairment. An adequate reconstruction therefore calls for the compensation of the expected growth deficit to achieve symmetry and an optimized function of the extremity. Between 1975 and 2000, 55 patients (24 female, 31 male) were referred and treated at our institution. All patients were not older than 10 years (4.2-10, mean: 7.9 years). Histological diagnosis was Ewing's tumors ( n=19) and osteosarcoma ( n=36). Localization in the extremities was predominantly in the lower extremity with 63.6% around the knee joint (distal femur and proximal tibia). Surgical treatment was performed in all patients. Endoprosthetic reconstruction was performed in 34 patients (lower extremity n=30, 29 Howmedica Modular Reconstruction System, HMRS, 1 Pafford prosthesis; upper extremity n=4, 4 HMRS); in another case of humeral tumor involvement a resection replantation procedure was carried out. The other patients received a rotationplasty ( n=7), a biologic reconstruction ( n=6), resection replantation of the humerus ( n=1), no reconstruction ( n=3), or had to be amputated ( n=4). After a mean follow-up of 114 months (24-256 months) 11 patients had died. Even in young children endoprosthetic reconstruction with relatively early mobilization and high quality of life can be a very successful method for limb salvage. Optimal reconstructive results following resection of primary malignant bone tumors in children are reached when indications and limitations of each method are carefully considered on an individual basis taking into account the patients' expectations.

Activities of Daily Living↗

[Surgical management of skeletal metastases of the extremities].

Operations on skeletal metastases of the extremities represent the most frequent surgical treatment in orthopedic oncology. From 1970 to 1997, 340 patients were operated on in 383 surgical procedures in the Orthopedic Department of the University of Heidelberg. Carcinoma of the breast and renal cell carcinoma were the most common primary tumors. Operations concerned mainly the femur with acetabulum (55%) and the humerus with glenoid (25%). A pathologic fracture occurred in 51%. While in these cases surgical treatment is absolutely necessary, it is contraindicated in preterminal patients. In imminent fractures or peripheral compression syndromes, the indication is relative and has to be justified on a multidisciplinary basis. Marginal resection of the tumor is allowed because of the limited life expectancy of most patients. Only in patients with solitary metastases and a better prognosis the resection of metastasis margin can be more extensive. Intensification of local therapy by adjuvant irradiation is indicated in an R2 situation, depending on the patient's prognosis. An analysis of our cases from 1994 to 1996 exemplifies the increasing use of modular tumor endoprostheses (52%). In meta-diaphyseal and diaphyseal cases, cement-augmented osteosynthesis is mainly used (34%). The application of these established reconstruction techniques after metastasis resection in cases with adjuvant radiotherapy assures a pain-free extremity capable of weight bearing.

Bone Neoplasms↗

[Differential diagnosis of bone and soft tissue tumors by MRI].

Musculoskeletal neoplasms are rare, and both the medical history and complaints of the patients are usually uncharacteristic and of limited information. After a clinical evaluation and biplane conventional radiography, the clinician must classify the patient as having a nonprogressive or progressive primary benign, primary malignant, or metastatic bone tumor. In the case of a probably benign, nonprogressive bone tumor, the patient has to be observed continuously or an additional biopsy should be performed. In the case of a probably malignant lesion, the patient should be referred for further staging and treatment to an orthopedic oncologist. Conventional biplane radiography, scintiscan, computed tomography scan, and magnetic resonance imaging (MRI) are indispensable in staging and treatment planning for patients with musculoskeletal tumors. For limb salvage procedures, delineation of the tumor from adjacent tissue structures is crucial. Hence, MRI of the entire anatomic structure involved, together with adjacent joints, is of the utmost importance, both in the coronal and axial planes. The significance of MRI in clinical follow-up depends on keeping the sequences and imaging planes used constant. Differentiating pseudotumors from true neoplasms still poses a challenge. The cellular pattern and matrix characteristics of a lesion cannot definitely be identified as neoplastic even with application of all imaging modalities including MRI. Information on epidemiology, clinical picture, radiology, and histology of the lesion is necessary to draw a firm conclusion. Biopsy is still the first choice in making the diagnosis.

Adult↗

Percutaneous radiofrequency thermocoagulation of osteoid osteomas: factors affecting therapeutic outcome.

OBJECTIVE: To examine factors which affect local recurrence of osteoid osteomas treated with percutaneous CT-guided radiofrequency thermocoagulation. DESIGN AND PATIENTS: A prospective study was carried out on 45 patients with osteoid osteoma who underwent percutaneous radiofrequency thermocoagulation with a minimum follow-up of 12 months. RESULTS: There were seven local recurrences (16%); all occurred within the first year. Local recurrence was significantly related to a non-diaphyseal location (P<0.01). There was no significant relationship (P=0.05) between local recurrence and age of the patient, duration of symptoms, previous treatment, size of the lesion, positive biopsy, radiofrequency generator used or the number of needle positions. There were no complications. CONCLUSIONS: Osteoid osteomas in a non-diaphyseal location are statistically more likely to recur than those in a diaphyseal location when treated with CT-guided percutaneous radiofrequency thermocoagulation. This relationship between local recurrence and location has not been previously reported.

Adolescent↗

De novo malignant transformation of giant cell tumor of bone.

Two cases of malignant lesions are reported, both of which arose secondary to an originally benign giant cell tumor (GCT) of bone. The first case was a typical benign GCT, which occurred in the left proximal tibia of a 31-year-old woman. The tumor was treated by curettage and bone grafting. However, it recurred twice during 15 years of follow-up. The second recurrence showed that the lesion histologically had turned into malignant fibrous histiocytoma. The second case also started as an initially benign GCT that arose in the left distal femur of a 41-year-old man. The patient underwent curettage and bone grafting. The lesion recurred 13 years postoperatively. The histological appearance of the recurrent tumor showed it to be an osteogenic sarcoma. In both patients, radiation and never been given. Malignant transformation has rarely been reported in patients with GCT of bone who have not received radiation treatment.

Adult↗

Excision curettage and allografting of giant cell tumor.

Between 1987 and 1994 we followed 22 patients with giant cell tumors involving the long bones. Their average age was 31 years (range 17-50 years). Five patients had grade II tumors and the other 17 grade III lesions. The average volume of lesions after curettage was 231 ml (range 56-450 ml). All of the patients underwent a modified excisional curettage, and the cavity was filled with deep-frozen allogenic corticocancellous bone graft with supplementary fixation. Two patients developed postoperative complications including a superficial wound infection in one case and a traumatic tibial plateau fracture in one case. The overall outcome was good or excellent in 91% of the patients (i.e., 20/22 cases). There was no degenerative joint arthritis and, surprisingly, no instance of tumor recurrence. Allograft infection and fracture were not present. An allogeneic cortical strut with cancellous bone graft can be used safely and is effective for grafting cavitary lesions created after complete removal of the tumor.

Adolescent↗

[Extraneural metastasis of brain and spinal cord tumors. Report of 2 cases].

This case report refers to two patients with the rare entity of an extraneural metastasizing central nervous system tumor. The first patient presented with ipsilateral cervical lymph node metastases 4 years after a diagnostic biopsy and 2 years after removal of an anaplastic oligodendroglioma, respectively. The origin of the metastases was confirmed by immunohistochemistry. The second case described was found in a 26-year-old female who had suffered from a spinal cord pilocytic astrocytoma in infancy and been treated by surgery and radiotherapy. On postmortem examination, transformation to a primitive neuroectodermal tumor was found. Morphological and immunohistochemical features of the metastases were exactly identical with those of the primary tumors in both cases. The pathomechanisms of metastasizing CNS tumors are discussed with reference to the reasons why such cases are so rarely observed. The incidence of remote metastases and differences depending on tumor type are estimated. No relation to malignancy grade can be detected.

Adolescent↗

Immune responses to osteoarticular allografts of the knee--cytokine studies.

Immunological behaviour in correlation with bone allograft survival was studied in peripheral blood and synovial fluid from seven patients who had undergone large bone resection and allograft transplantation of the knee. Plasma and synovial fluid samples for cytokine measurements [interleukin (IL-1beta, IL-6) and tumour necrosis factor alpha (TNF-alpha)] were drawn from peripheral blood for diagnostic arthrocentesis. Two patients were monitored using consecutive sampling up to 12 months postoperatively. Graft survival was considered excellent, but local or diffuse resorption and also fatigue fractures were seen. These findings show that soluble products of T-cell, macrophage and osteoblast origin, produced as a response to the bone-graft antigens, might be responsible for the bone resorption seen in our material. The elevated IL-1beta and TNF-alpha levels detected support this statement.

Adult↗

Extraskeletal myxoid chondrosarcoma with multiple skeletal metastases.

Pulmonary metastases are not unusual in extraskeletal myxoid chondrosarcoma; however, only two patients have been reported with multiple bony metastases. We report here one patient with extraskeletal myxoid chondrosarcoma associated with lung and multiple bony metastases. After chemotherapy, the primary lesion was resected, but lung and multiple bony metastases were found 20 months later. The bony metastases were in the right femur, right humerus, and at multiple vertebral levels. Because of a pathologic fracture of the right femur, the metastases in the right femur and right humerus were surgically stabilized. After chemotherapy, the lung metastases were resected, and those in the vertebral bodies were treated with radiotherapy.

Bone Neoplasms↗

Characterisation of complex karyotype changes in two canine bone tumours.

Two dogs developed osteosarcomas. In both cases, the cytogenetic analysis revealed the presence of numerically and structurally altered karyotypes. The chromosome ranges were 90 to 99 and 65 to 67, respectively. Centric fusions frequently identified were 1/3, 1/30 and 2/9 in one case, 3/19, 12/18 and 13/17 in the other.

Animals↗

Intraosseously transplantable osteosarcoma with regularly disseminating pulmonary metastases in rats.

An intratibially transplantable osteogenic sarcoma in rats, that regularly disseminates osteogenic metastases in the lung, is described. The tumor take rates exceeded 90% 30 days after transplantation. Pulmonary metastases were diagnosed in about 99% of the animals, while metastases in the kidneys (14%), lymph nodes (13%) and liver (3%) occurred less frequently. The mean survival time of untreated animals varied between 36 and 48 days, depending on the age at transplantation. Histologic and scintigraphic findings are reported.

Animals↗

Benign bone tumors.

There is little information in the literature concerning the role of bone scanning in benign bone neoplasms except for sporadic reports. Since the advent of 99mTc-polyphosphate, bone imaging has proven feasible and useful in locating the cause of bone pain, such as in osteoid osteomas, which are not always radiologically apparent, and in evaluating whether or not a radiologic lesion is indeed benign and solitary. Blood-pool images are particularly important in neoplastic disease, since the absence of hyperemia in the immediate postinjection period favors the diagnosis of a benign neoplasm, as does low-grade uptake on the delayed study. The scan, including pinhole magnification images, is especially valuable in diagnosing lesions in the spine and pelvis, which are poorly seen radiologically. We have studied various types of benign bone tumors, including simple and aneurysmal bone cysts, fibrous cortical defects, and nonossifying fibromas, all of which had minimal or no increased uptake of the radiopharmaceutical, unless traumatized. Although osteochondromas and enchondromas showed varied accumulation of activity, the scan was useful in differentiating these from sarcomatous lesions. All osteoid osteomas demonstrated marked activity, and could be accurately located preoperatively, as could the extent of fibrous dysplasia. The bone scan in the reticuloses also showed abnormal accumulation of activity, and aided in arriving at the prognosis and treatment of histiocytic bone lesions.

Bone Cysts↗

Rehabilitation after total femur replacement: a report of 2 cases.

We describe the rehabilitation of 2 patients who underwent total femur replacement after neoplastic involvement of the bone. In the past, patients with cancer of the femur were generally advised to undergo a hip disarticulation or transfemoral amputation. It is now feasible to salvage the limb in selected patients, by excising the entire femur together with any contiguous soft tissue tumor and replacing it with an endoprosthesis. The surgical literature contains a number of reports on total femur replacement, which mention the rehabilitation aspects only briefly, but we found nothing on this relatively uncommon form of surgery in the rehabilitation literature. Physiotherapy techniques such as active assisted exercises, isometric exercises, and hydrotherapy are extremely useful during the early phase of rehabilitation to facilitate a graduated strengthening program. Certain exercises, such as active hip abduction or knee flexion, may not be permitted for several weeks to protect muscles that have been reattached to the prosthesis. Partial weight bearing may be required to allow incorporation of bone allograft around the prosthetic hip joint. Because of these factors patients with total femur replacement may need a longer period of rehabilitation (6-8wk) than patients with total hip or knee replacement. Patients with total femur replacement can, however, achieve full independence with an appropriate rehabilitation program.

Activities of Daily Living↗

Bizarre parosteal osteochondromatous proliferation (Nora's lesion): a retrospective study of 12 cases, 2 arising in long bones.

Twelve cases of bizarre parosteal osteochondromatous proliferation (BPOP), also known as Nora's lesion, are reported. Ten lesions were located in the small bones of the hands, and 2 were located in long bones (femur and proximal tibia). Patient age ranged from 12 to 63 years (average, 30.3 years). Radiography of the lesions in the hand bones showed calcific masses attached to the underlying cortex, without interruption of the latter. The long bone lesions revealed unusual findings. In the femur, BPOP presented with extensive cortical destruction and was suggestive of a malignant lesion. This presentation has not been described to date. In the tibia, the lesion was located in the soft tissue without cortical attachment. This type of BPOP probably represents an immature lesion that over time will mature to solid cortical attachment. On histologic examination, all lesions demonstrated 3 distinct components with variable degrees of representation: (1) hypercellular cartilage with calcification and ossification, with the calcified cartilage having a characteristic basophilic tinctorial quality; (2) cancellous bone undergoing maturation; and (3) spindle cell stroma without cytologic atypia. In 1 case with a long-standing history, the cartilaginous component was minimal. BPOP, together with florid reactive periostitis and turret exostosis, may represent different stages in the development of a posttraumatic proliferative process. BPOP apparently arises from the periosteal tissues through a process of cartilaginous metaplasia.

Actins↗