Osteosarcoma of the pelvis: experience of the Cooperative Osteosarcoma Study Group. (33/407)

PURPOSE: To define patients and tumor characteristics as well as therapy results, patients with pelvic osteosarcoma who were registered in the Cooperative Osteosarcoma Study Group (COSS) were analyzed. PATIENTS AND METHODS: Sixty-seven patients with a high-grade pelvic osteosarcoma were eligible for this analysis. Fifteen patients had primary metastases. All patients received chemotherapy according to COSS protocols. Thirty-eight patients underwent limb-sparing surgery, 12 patients underwent hemipelvectomy, and 17 patients did not undergo definitive surgery. Eleven patients received irradiation to the primary tumor site: four postoperatively and seven as the only form of local therapy. RESULTS: Local failure occurred in 47 of all 67 patients (70%) and in 31 of 50 patients (62%) who underwent definitive surgery. Five-year overall survival (OS) and progression-free survival rates were 27% and 19%, respectively. Large tumor size (P =.0137), primary metastases (P =.0001), and no or intralesional surgery (P <.0001) were poor prognostic factors. In 30 patients with no or intralesional surgery, 11 patients with radiotherapy had better OS than 19 patients without radiotherapy (P =.0033). Among the variables, primary metastasis, large tumor, no or intralesional surgery, no radiotherapy, existence of primary metastasis (relative risk [RR] = 3.456; P =.0009), surgical margin (intralesional or no surgical excision; RR = 5.619; P <.0001), and no radiotherapy (RR = 4.196; P =.0059) were independent poor prognostic factors. CONCLUSION: An operative approach with wide or marginal margins improves local control and OS. If the surgical margin is intralesional or excision is impossible, additional radiotherapy has a positive influence on prognosis.  (+info)

Tissue-specific RNA surveillance? Nonsense-mediated mRNA decay causes collagen X haploinsufficiency in Schmid metaphyseal chondrodysplasia cartilage. (34/407)

Mutations resulting in a premature termination codon (PTC) are a major cause of inherited disorders, and the majority of these mutant RNA transcripts are subjected to nonsense-mediated mRNA decay (NMD). This RNA surveillance results in reduced mutant allele expression, the extent of which can impact on the clinical severity. The molecular mechanisms of NMD in mammalian cells, its relationship to splicing and translation, downstream sequence elements and binding factors remains only partially understood. Currently there is little information on whether the extent of NMD is gene- or tissue-specific, although nonsense mutation inhibition of RNA splicing has been shown to exhibit some tissue and gene specificity in vitro. Schmid metaphyseal chondrodysplasia results from heterozygous mutations in the gene for collagen X (COL10A1), expressed by the hypertrophic chondrocytes of growth plate cartilage. In one patient a PTC mutation has been shown to result in complete NMD and collagen X haploinsufficiency in cartilage. Here we show that, in this patient, and in another with a different collagen X PTC mutation also leading to complete NMD in cartilage, the mutant mRNAs were not subjected to NMD in non-cartilage cells (lymphoblasts and bone cells). These data suggest that novel RNA surveillance mechanisms may exist in cartilage and that tissue specificity of NMD could be of importance in understanding the molecular pathology of nonsense mutations. Furthermore, the demonstration of collagen X haploinsufficiency in the second patient to be studied at the level of tissue expression, confirms that nonsense mutations leading to complete mutant collagen X mRNA degradation in cartilage is an important molecular cause of SMCD.  (+info)

Surgical reconstruction of late pelvic post-traumatic nonunion and malalignment. (35/407)

We have retrospectively reviewed the clinical and radiological results in 204 consecutive adult patients who had surgical correction of 70 late post-traumatic pelvic nonunions and 134 malalignments. The deformed pelvises were subdivided into united (true), unstable, ununited, and partially stable malalignments with heterotopic bone. The principal complaints were of pain, pelvic instability, sitting imbalance, and apparent limb-length discrepancy. After surgery, 195 patients (96%) achieved a primary union and 144 (71%) had slight, intermittent or no pelvic pain, while pelvic instability was entirely eliminated. Overall, 131 patients (64.2%) were extremely satisfied, 58 (28.4%) were satisfied and 15 (7.4%) were unsatisfied. After reconstruction of the malaligned pelvises, 67 results (50%) were anatomical, 47 (35%) were satisfactory and 20 (15%) were unsatisfactory. For a pelvic nonunion with local osteopenia and malalignment, stabilisation of all three pelvic columns is recommended. True pelvic (united) malunions were the most satisfactorily realigned and had the fewest complications. Ununited and unstable malalignments, especially those with heterotopic bone, had the poorest corrections and the most neurological complications. A therapeutic alternative, by the local resection of a symptomatic bony prominence, and fixation in situ of a posterior pelvic nonunion, gives highly effective symptomatic relief with fewer complications. Despite this, many patients had persistent low back pain.  (+info)

Childhood pelvic osteomyelitis presenting as a "cold" lesion on bone scan: case report. (36/407)

A case of occult pelvic osteomyelitis is presented. The involved portions of the left pubis and left ischium presented as "cold" areas on the original bone scan with 99mTc-diphosphonate. The presumed mechanism for this unusual finding in osteomyelitis is compression of the microcirculation to bone by subperiosteal and intraosseous pus.  (+info)

Iliosacral screw fixation of the posterior pelvic ring using local anaesthesia and computerised tomography. (37/407)

Various techniques have been used for the fixation of the posterior pelvis, each with disadvantages specific to the technique. In this study, a new protocol involving the placement of posterior pelvic screws in the CT suite is described and evaluated. A total of 66 patients with unstable pelvic ring injuries was stabilised under local anaesthesia with sedation. The mean length of time for the procedure was 26 minutes per screw. There were no technical difficulties or misplaced screws and no cases of infection or nonunion. All patients stated that they would choose to have the CT scan procedure again rather than a procedure requiring general anaesthesia. The charges for the procedure were approximately 1840 pounds sterling (2800 dollars) per operation. CT-guided placement of iliosacral screws is a safe, feasible, and cost-effective alternative to radiologically-guided placement in the operating theatre in selected patients.  (+info)

Linear sebaceous naevus syndrome and resistant rickets. (38/407)

The association between vitamin-D-resistant rickets and linear sebaceous naevus syndrome is extremely rare. Only eight cases have been described in the English literature and in none were the skeletal aspects addressed. We present three new cases and describe the musculoskeletal features. The details and outcome of surgery for correction of the deformities are discussed. The disturbances of metabolism of vitamin D and the effects of pharmacological treatment are also described.  (+info)

Endoscopic management for traumatic occlusion of posterior urethra. (39/407)

When traumatic posterior urethral transection occurs with serious pelvic fracture, the patient is usually under severe hemorrhagic shock and multiple injuries. It is of prime importance to treat shock and the associated injuries promptly. As for the urethral disruption a simple suprapubic cystostomy at the moment is preferred. Occlusion of the posterior urethra would eventually appear and can be managed endoscopically 3-6 months later. Fifteen such cases were thus managed from 1984 to 1990 with an improved endoscopic technique with a success rate of 93.3% (14/15). The advantages of this technique and the merits and demerits of various primary urethral reconstruction procedures are discussed.  (+info)

Biometry of the anterior border of the human hip bone: normal values and their use in sex determination. (40/407)

Sixteen different variables and 3 indices for the anterior border of 42 human hip bones from a Spanish skeletal collection were studied. Values for 15 of these variables and for the 3 indices are reported. We were unable to detect statistically significant differences between means relating to side in any of the variables and indices studied. Statistically significant differences were detected between means in relation to sex for 4 variables (distance from the anterior superior iliac spine to the pubic tubercle, distance from the anterior inferior iliac spine to the iliopubic eminence, distance from the anterior inferior iliac spine to the pubic tubercle, length of the notch between the anterior inferior iliac spine and the iliopubic eminence). These variables could be used for sex determination from the human hip bone or its fragments.  (+info)