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[Possibilities of endoprosthesis in osteogenic sarcoma in children].

Endoprostheses of the knee (14) and hip (after Sivash ) (2) joints were used in the treatment of 16 children operated on for osteogenic sarcoma at the Center Clinic. Postoperative complications leading to amputation occurred in two cases of lesions of the proximal part of shinbone . Good functional results were obtained: knee joints supplied with endoprosthesis flexed through 80 degrees. The results are only preliminary considering the relatively short period of the study but they are encouraging enough.

Adolescent↗

Radical resection and allograft replacement in the treatment of bone tumors.

Resection and allograft transplantation in the treatment of bone tumors was introduced in the United States by Parrish in the mid 1960's, based on the demonstration by several investigators that fresh frozen allografts were well tolerated by the host. Since 1971, we have performed 63 such procedures in 60 patients who have had segmental resections for malignant and aggressive bone tumors. Analysis of the results in 46 patients who have been followed longer than 1 year (mean followup of 2.7 years) show that none of the patients have an identifible immunologic reaction to the graft. Twenty-five (53.5%) of the patients had an uneventful recovery and achieved functional status without complication ("good" resuls). An additional eleven patients (23.9%) who exhibited late complications of allograft fracture, infection, or nonunion required further surgery to achieve the same degree of functional restoration as the former group ("satisfactory" results), thus raising the total of good and satisfactory results to 78.2%. Seven of the patients (15.2%) were gross failures in that two required amputation, two had the grafts removed and three have died. Although these data suggest that the procedure is a successful one for treatment of certain types of bone tumors, continued follow-up is required to ascertain the long term results. The technique should be reserved for patients with aggressive or low grade tumors, in which resection can be performed without excessive tissue sacrifice and who will not require adjuvant chemotherapy.

Adolescent↗

Extendable tumour endoprostheses for the leg in children.

From 1986 to 1991 we fitted 20 children with endoprostheses after resection of malignant bone tumours of the leg; six have reached skeletal maturity and are the subject of this study. Reconstruction of defects in growing limbs in which the eventual shortening can be predicted requires the use of extendable prostheses. The mean age at operation was 11 years (9.2 to 13.7) and the average follow-up period was 6.3 years (4.3 to 7.6). The diagnosis was osteosarcoma in five patients and Ewing's sarcoma in one. All tumours were Enneking stage-IIB. When seen for follow-up all patients were free from disease. The extendable implants used included the Pafford-Lewis prosthesis and the Kotz Modular Femur Tibia Reconstruction system with a compatible, newly-designed growth module. Telescope-like elongation of the prostheses was performed by insertion of a screwdriver through a small skin incision. Active epiphyseal growth in the adjacent growth plate was preserved by using prosthetic stems with a smooth surface. The mean length gained was 13.15 cm (4.5 to 19.5) requiring 53 planned procedures. Seven revision operations were necessary for complications. Functional evaluation showed excellent and good results in all cases. Stress-shielding at the site of anchorage of the prosthesis was more pronounced than in adults. Implantation of extendable endoprostheses in children provides a reasonable alternative to rotationplasty, but limb salvage requires more operations.

Adolescent↗

Limb-salvage for osteosarcoma.

Limb-salvage surgery plays an important role in the treatment of osteosarcoma. Among 104 patients with osteosarcoma we managed from 1974 to 1993, 60 were treated with limb-salvage procedure. 44 patients with IIB tumor received adjuvant chemotherapy, and the five-year survival rate was 46.69%. The local recurrence rate of these 60 patients was 15%, the infection rate 11.7%, and the prosthetic mechanical complication was 13.3%. Our results suggest that limb-salvage procedures are not only possible but also can provide patients with better function.

Adolescent↗

Technetium-99m labeled agents for skeletal imaging.

Technetium-99m labeled radiopharmaceuticals are the currently accepted agents of choice for skeletal imaging. Their introduction in 1971 literally initiated a new era in clinical bone scanning. The development of techniques for reducing Tc(VII) with Sn(II) provided the means for complexing this useful radionuclide with various phosphorus-containing compounds which were already known to be avid bone seekers. Long chain polyphosphates were widely used at first, but have been superceded by pyrophosphate and its organic analogs, the diphosphonates. Pyrophosphate is characterized chemically by P--O--P bonds, and the diphosphonates by P--C--P bonds. The chemical forms of their complexes with tin and technetium are not known, but they behave in many respects as weak chelates. Labeling efficiencies for 99mTc of 95% or better are routinely obtainable with both "in house" preparations and commercial kits. Proper molar concentrations and ratios of phosphorus-compound to tin are necessary for both for good labeling and to achieve optimum tissue distribution. Unreacted TcO4- and reduced unbound 99mTc are both potential contaminants in these preparations and must be considered in radiochemical quality control. In vivo tissue distribution and kinetics of the 99mTc-Sn-phosphorus compounds differ with details of preparation, category of agent, and clinical status of the patient. Blood clearance is multi-exponential, with skeletal uptake and urinary clearance accounting for most of the activity. Scanning may be started in 2 1/2 to 4 hr, at which time skeletal activity is on the order of 40 to 50% of the injected dose. The primary indication for bone scanning remains the detection of metastases from extraskeletal malignancies, and the 99mTc labeled agents are more sensitive than either radiographs or Fluorine-18 for demonstrating active lesions. In addition, many new applications in evaluating benign bone disease have widened the clinical scope of skeletal imaging which is rapidly becoming one of the most important studies in nuclear medicine.

Bone Diseases↗

Evaluation of hip disorders by radiography, radionuclide scanning and magnetic resonance imaging.

Thirty-six hips were studied because of significant hip pain. Radiography of the hip and bone scintigraphy showed subtle changes. Magnetic resonance imaging (MRI) was performed using a 1.5 Tesla superconductive unit. All MRI findings were confirmed by surgical or pathologic results. Twenty-nine hips had a single lesion, including: infection (one), fracture (eight), avascular necrosis of the femur(two), or contralateral hip (four), transient osteoporosis (six), osteoporosis (one), post-irradiation myositis (one), metastasis (four), and synovitis (two). Twenty-six lesions (89.6%) appeared normal on the radiographs of the hip, while three lesions (10.4%) showed only osteoporotic change. Another seven hips had more than one lesion, including: avascular necrosis and fracture (four), fracture foci (two), and metastasis and fracture (two). Radiography of the hip showed either a negative finding or detected only a single lesion, missing other important pathologic foci. MRI is extremely sensitive to alterations in the bone marrow that may represent pathology occult to plain radiography and bone scintigraphy of the hips. For diagnosis and treatment planning, MRI of the hips should be performed early in patients with persistent pain and negative radiography findings.

Acetabulum↗

[Osteosarcoma cell immunophenotype and heterogeneity].

48 cases of osteosarcoma, including 18 cases of osteoblastic type, 16 of fibroblastic type and 14 of chondroblastic type, were studied in accordance with Dahlin's classification. All specimens were stained with 11 specific markers using immunohistochemical ABC methods. Positive immunostaining for BMP was found in all of these cases. The results showed 48 vimentin positive cases, 46 actin positive cases, 5 keratin positive cases and 4 desmin positive cases. Neither cytokeratin nor EMA was identified in any of the cases. Positive reactions to S-100 protein, collagen type IV, UEA-1 and factor XIII were observed in 26, 20, 36 and 13 of the cases respectively. The results of this study confirm that osteosarcoma possesses the unique multidirectional differentiation potential toward osteoblastic, chondroblastic, myofibroblastic, fibrohistiocytic and epithelial cells, and also indicate that immunophenotypic analysis is useful for the diagnosis, differential diagnosis and classification of the tumor cells of osteosarcoma.

Actins↗

[Aggressive fibromatosis of bone].

Twenty four cases of pathologically diagnosed aggressive fibromatosis of bone were reported. 17 patients were followed for at least 2 years. Among them 4 recurred and the histological findings of their lesional tissues obtained after recurrence remained benign; one case refused radical treatment and the second biopsy done at 3.5 years after the appearance of the tumor showed no malignant changes; but another case died of lung metastasis. The authors suggest that the criteria for final diagnosis of an aggressive fibromatosis of bone are as follows: Besides the pathological findings, the lesion does not recur or metastasize more than 2 years after operation or it does recur, but still shows benign features histologically. As for treatment, marginal or wide excision is the procedure of choice.

Adolescent↗

[Rotation grafts].

Nowadays rotationplasty is well established as an acceptable procedure for limb salvage in patients who have a malignant tumor in the femur or tibia. The main indication is that it is the alternative to amputation. Rotationplasty should be used in very young children because of the growth-dependent complications that can be expected after tumor resection. The different types of rotationplasty, the operative technique, prosthetic care, and the results of 104 patients are shown.

Adolescent↗

Assessment of patient function after limb-sparing surgery.

Cancer rehabilitation is becoming more of a focus for the field of physiatry due to increased longevity and the side effects of treatment. In order to investigate the rehabilitation needs of patients undergoing limb-sparing procedures, chart analysis was conducted on 17 children treated for primary bone tumors by resection and an expandable endoprosthetic replacement. Each patient underwent a course of postoperative inpatient and outpatient physical therapy and was followed over an average of 2.5 years. Gait training was relatively straightforward and in seven patients required neither orthosis nor ambulatory aid. The other ten patients walked with a knee orthosis, axillary crutches, or both. Until the time came for reoperation to lengthen the implant, a shoe lift of 1in maximum was added to compensate for the limb length discrepancy. These findings compare favorably with the more complex requirements of high proximal amputees with external prostheses, including more difficult gait training and the need for frequent adjustments, as well as prosthetic replacement as the children grow. It is clear that children undergoing limb-sparing surgery have special needs that should be addressed, including early mobilization, gait training, adjustment to repeated brief hospitalizations for lengthening, and continued follow-up to monitor their activity restriction.

Adolescent↗

[The treatment and prosthesis after an interilioabdominal amputation for sarcomas of the bones and soft tissues].

According to data of the Research Institute of Oncology named after N. N. Petrov, progress was achieved in treatment of patients who were subject to interilioabdominal amputation for malignant tumors. Five years survival after such operations was 19.7 +/- 3.4%. The prosthesis for patients subjected to interilioabdominal amputations was developed in Research Institute of Prosthesis (Saint Petersburg). It was successfully used in 11 patients in the age from 8 to 51 years. The original construction of the prosthesis gives the patients possibility to walk without crutches, move along staircases with 25-cm-high footsteps and to use urban transport.

Adolescent↗

Thallium-201 scintigraphy to assess effect of chemotherapy in osteosarcoma.

UNLABELLED: Imaging results in patients with high-grade osteosarcoma of the extremities were reviewed to determine whether scintigraphic appearance correlated with histologic response to preoperative chemotherapy. METHODS: Histologically, the percent tumor necrosis in specimens from 30 patients were classified into three grades: grade 1 = necrosis less than 60%, grade 2 = 60%-89% necrosis and grade 3 = diffuse necrosis greater than 90% based upon whole transverse sections. Scintigraphically, we analyzed 201TI uptake before and after preoperative chemotherapy. The changes in the tumor-to-background ratio were defined by an alteration ratio. RESULTS: Of the 11 patients with a grade 1 response, the ratio showed -67.1% +/- 45.4% (mean +/- s.d.). Of the 9 patients with a grade 2 response, the ratio showed 37.9% +/- 29.9% of the 10 patients with a grade 3 response the ratio showed 105.5% +/- 12.4%. The ratios correlated well with the histologic grades (p < 0.0001; analysis of variance). CONCLUSION: Thallium-201 scintigraphy accurately assesses the effect of chemotherapy on osteosarcoma.

Adolescent↗

Reconstruction by Grosse-Kempf nailing in resections for bone tumor.

The authors report 38 reconstruction by Grosse-Kempf nailing for resection of bone tumor; there were: intercalar resections: 16; arthrodeses of the knee: 13; arthrodesis of the ankle joint: 5; gyroplasty: 4. Massive homoplastic bone graft was used in 26 cases, in 3 of which autoplastic grafting was associated; autoplastic grafts were used exclusively in 7 cases, and a cement spacer was used in 1. Twenty-nine patients (76%) obtained satisfactory final results, but in 10 cases secondary surgery was performed for a total of 14 operations, of which 7 major (1 nail substitution and 6 vascularized fibula operations). There were 7 failures: mechanical complications: 6; infection: 1. The highest number of non-unions were observed where massive homoplastic grafting was used: intercalar reconstruction: 11; arthrodesis of the knee: 7. The results were evidently better and the incidence of complications low in cases in which autoplastic grafting was used or where gyroplasty was performed. Absolute stiffness and best possible contact are difficult to obtain with blocked intramedullary nailing. On the contrary, when autoplastic grafts are used or when gyroplasty is performed micromovements allowed the instrumentation are compatible with good consolidation.

Adolescent↗

[Low grade intramedullary osteosarcoma. Apropos of 8 cases].

PURPOSE OF THE STUDY: The authors reviewed eight patients treated for Low Grade Intramedullary Osteosarcoma (LGIO). They analyzed the radiographical and histological features and evaluated the prognosis of this disease. MATERIALS: Eight cases of LGIO were studied. The mean follow-up period was 3.9 years. LGIO is defined as a Broders classification grade I osteosarcoma. The study involved 3 males and 5 females. Mean age at the time of diagnosis was 28. METHODS: Clinical records, radiographs and C.T. Scan were available and reviewed in all cases. Five patients had had MRI. In addition to the conventional histological study, ultrastructural studies were conducted in three of the cases. On their last examination, all patients received a clinical and X-ray assessment. RESULTS: The tumor was localized around the knee in six cases on the humerus and on the proximal femur in one case. The tumor was situated in the metaphysis and was most often lytic. A malignant tumor was suspected at the outset for only two of the patients. The scanner and the MRI revealed a cortical discontinuity and extraosseous involvement in five cases. Seven patients had a wide resection, which was intra-tumoral once. The histological examination of two of those resections revealed limited areas of anaplasia; complementary chemotherapy was then conducted. We did not observe any local recurrence in this group. We observed, however, an iliac metastasis, for which another wide resection was performed. This last patient remained free of disease three years later. One patient had radiotherapy and chemotherapy; he died from pulmonary metastasis after his LGIO turned into high grade osteosarcoma. DISCUSSION: LGIO are rare, they represent 1.8 per cent of all Osteosarcomas in the Cochin file. The mean age of patients contracting LGIO is higher than that of conventional osteosarcoma patients. Observation of the radiographs and histological examination could lead to think the tumor is benign, and mistake it with a fibrous displasia. The C.T. Scan and the MRI have the advantage of showing soft tissue invasion and help the histological examination. The wide resection is the only treatment able to cure the patient. Chemotherapy would be useful for LGIO cases containing limited arcas of anaplasia. CONCLUSION: LGIO is a slowly-evolving tumor. It remains localized for a long period of time. The histological diagnosis is difficult and requires a pathologist familiar with bone tumors. Its treatment requires surgery. LGIO must be distinguished from conventional osteosarcomas because of its better prognosis.

Adult↗

[Surgical management of bone metastases of the lower extremity with AO interlocking nail].

Between January 1991 and June 1995 we have operated on 19 patients (9 male, 10 female) with 22 skeletal metastases of the lower limb (19 femora, 3 tibiae) using a static interlocking nail. Closed intramedullary nailing without resection of the metastasis has been established as our standard procedure. We have stabilized 15 patients with advanced osteolysis and seven pathological fractures. Sixteen patients underwent postoperative local radiation therapy with 40 Gy. As intraoperative complications we have observed one fracture of an osteolysis and one death due to fat embolism. Postoperatively there were observed one seroma, one haematoma and one patient with non fatal pulmonary embolism following DVT. Two patients died within the first 30 postoperative days because of tumor progression. All patients surviving longer than 30 days could be mobilized under full weight-bearing. Morphine like analgetics for metastasis related pain were no longer needed. A secondary instability has not been observed within a mean survival time of 199 days (811 longest follow up). Closed intramedullary nailing in combination with postoperative local radiation therapy seems to be an appropriate and technically non demanding procedure to stabilize skeletal metastases of the lower limb in patients with a short or medium-term expectation of life.

Aged↗

CD3- CD56+ non-Hodgkin's lymphomas with an aggressive behavior related to multidrug resistance.

CD56 expression has been reported previously in some non-Hodgkin's lymphoma (NHL) characterization. They principally involve the nasopharynx, are related to Epstein-Barr virus (EBV), and may be classified as either T- or non-T-natural killer (NK) cells according to CD3/T-cell receptor (TCR) status at the genomic or protein level. The present study reports three cases of non-nasal NK-NHL with the following characteristics: an agressive clinical behavior, heterogenous morphological data evoking pleomorphic T-cell malignant lymphoma, a non-T-NK phenotype using flow cytometry, and immunochemistry. The three cases were CD56+ without membrane expression of specific T markers (CD3, CD5, and TCR). Heterogenous results were observed concerning different antigens: CD2, CD4, CD8, CD16, CD94, CD122, TiA1, perforin, and granzyme B. There was no evidence of detectable clonal TCR gene rearrangement with polymerase chain reaction. No NK activity was detected in the two tested cases, and no relation was found with EBV. Multidrug resistance investigations suggest that agressive clinical findings could be related to MDR1 gene expression as confirmed by MDR1 mRNA detection, MDR1 gene product (Pgp) expression, and a functional multidrug resistance study using rhodamine efflux by flow-cytometry.

ATP Binding Cassette Transporter, Subfamily B, Mem↗