Dedifferentiated chondrosarcoma in Albright syndrome. A case report and review of the literature.
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Bone Neoplasms↗
Biomedical subjects
S Blasius
Publications and source records attributed to S Blasius.
Ewing's sarcoma of the femur. Prognosis in 69 patients treated by the CESS group.
We reviewed the treatment outcome of 69 patients with Ewing's sarcoma of the femur. The patients received chemotherapy according to the CESS 81 (n 14), CESS 86 (n 43), and CESS 91P (n 12) protocols. The 10-year relapse-free survival rates were 36%, 65%, and 65% (p = 0.01). 68 patients received local treatment. The primary tumor was treated by surgery without radiotherapy in 28 patients; 1 developed a local recurrence and 7 metastases. 10 patients received radiotherapy alone; 4 developed metastases and 4 local recurrences and metastases. 30 cases had a combination of surgery and radiotherapy; 7 developed metastases and 1 a local recurrence and metastasis. The survival of patients after radiotherapy alone was worse than that of patients after surgery with/without radiotherapy (p = 0.005). Pathological fractures (n 16) did not influence the prognosis.
Adolescent↗
[Angiosarcoma of the hand. Case report].
Angiosarcoma is a very rare malignant bone tumour. The case of a 45-year-old man is presented with a primary epithelioid hemangioendothelioma in the fourth metacarpus. After radical extirpation of the tumour radiation followed. The function of the hand was fully preserved. Five years after operation the patient is free of disease.
Bone Neoplasms↗
Significance of surgical margin on the prognosis of patients with Ewing's sarcoma. A report from the Cooperative Ewing's Sarcoma Study.
BACKGROUND: There is little information regarding an adequate surgical margin for local control of Ewing's sarcoma. METHODS: Two hundred and forty-four patients (PTS) with Ewing's sarcoma who were registered in the Cooperative Ewing's Sarcoma Studies underwent surgical treatment. Ninety-four PTS underwent definitive surgery (surgery alone), 131 PTS received postoperative irradiation, and 19 PTS received preoperative irradiation. The surgical margins were distributed as follows: radical, 29 PTS; wide, 148 PTS; marginal, 39 PTS; and intralesional, 28 PTS. The impact of the surgical margin on the treatment outcome of PTS was analyzed statistically. RESULTS: The local or combined (local recurrence and systemic metastasis) relapse rate after surgery with or without irradiation was significantly lower compared with that after definitive irradiation (irradiation alone) (7% vs. 31%, P < 0.0001). The local or combined relapse rate after complete resection (radical or wide margin) with or without irradiation was less compared with that after incomplete resection (marginal or intralesional margin) with or without irradiation (5% vs. 12% P = 0.0455). The local or combined relapse rate did not greatly decreased after irradiation after incomplete surgery (from 14% to 12%). In both groups of good (viable tumor cells < 10%) and poor (viable cells > or = 10%) histologic response, the difference in systemic or combined relapse rate between patients undergoing complete and incomplete surgery was not significant. The 10-year overall survival of the PTS for each of the margins was distributed as follows: radical, 58%; wide, 65%; marginal, 61%; and intralesional, 71% (P = not significant). CONCLUSIONS: Surgery in patients with Ewing's sarcoma adds to the safety of local control. Under the current treatment regimen with intensive chemotherapy and irradiation, complete resection of the tumor appears capable of decreasing the risk of local recurrence.
Adolescent↗
Accuracy of pedicle screw placement in lumbar vertebrae.
STUDY DESIGN: The location of pedicle screws (n = 42) in four human specimens of the lumbar spine and in 30 patients (n = 131 screws) after lumbar spinal fusion was assessed using computed tomography. OBJECTIVES: To determine the accuracy of pedicle screw placement in lumbar vertebrae and the reproducibility and repeatability of the computed tomography examination. SUMMARY OF BACKGROUND DATA: Failures in the placement of transpedicular screws for lumbar fusion are reported. The evaluation of such screws using computed tomography examination has not been investigated. METHODS: After surgery, the specimens were dissected in transversal slices to observe macroscopically the location of the pedicle screw and to correlate these observations with the computed tomography images. All patients were examined by one observer. To determine the reproducibility and repeatability of the computed tomography examination, two observers studied computed tomography images of 12 patients (n = 58 screws) twice within 3 months. RESULTS: In the specimens, 10 screws were observed to penetrate the medial wall of the pedicle. This correlated fully with the images. In the patients' group, 40% of all screws penetrated the cortex of the vertebra. Of all screws, 29% penetrated the medial wall of the pedicle. From the computed tomography images, it appeared that a deviation of more than 6 mm medially was a high risk for nerve root damage. Three months after his first examination, Observer 1 documented a different position in three of 58 screws (kappa = 0.90). Observer 2 found a different position in eight screws (kappa = 0.65). The comparison between the reviews of the two observers showed a different opinion for the first evaluation, four disagreements (2-4 mm) and 17 disagreements (0-2 mm; kappa = 0.34), and for the second evaluation, four disagreements (2-4 mm) and 12 disagreements (0-2 mm; kappa = 0.43). CONCLUSIONS: Correct placement of transpedicular screws for spinal fusion seems to be more difficult than it looks. The computed tomography scanning is useful for differential diagnosis of postoperative radicular syndromes after lumbar transpedicular fixation.
Bone Screws↗
Influence of intralesional surgery on treatment outcome of chondrosarcoma.
BACKGROUND: Intralesional (IL) surgery for chondrosarcoma is an inadequate procedure; however, sometimes it cannot be avoided. The outcome of 26 patients (PTS) who underwent IL surgery for chondrosarcoma was analyzed. METHODS: Median follow-up time was 146.5 months. Eighteen tumors were located in the axial skeleton and 8 in the extremities. The histologic grade distribution of the chondrosarcoma was: 14 Grade 1, 8 Grade 2, and 4 Grade 3. Diagnostic problems were noted in 4 of the 18 central tumors and 7 of 8 extremity tumors. RESULTS: The 20-year relapse free survival rate for the 26 PTS who underwent IL surgery was 7% and was 64% for 38 PTS who underwent wide or marginal surgery (P = 0.03144). Conversely, the 20-year overall survival rate for the 26 PTS after IL surgery was 68% and was 66% for 38 PTS who underwent wide or marginal surgery (P = 0.03144). Conversely, the 20-year overall survival rate for the 26 PTS after IL (P = 0.2266). The overall survival rates for the PTS with tumors of histologic Grade 1 and 2 or 3 tumor was 85% and 44%, respectively (P = 0.02817). The overall survival rates for PTS with central tumors and extremital tumors were 57% and 87%, respectively (P = 0.0968). CONCLUSIONS: IL surgery for chondrosarcoma often leads to local relapse. However, local relapse does not always result in metastases and death. The prognosis for PTS after IL surgery tends to differ according to tumor grade. After local relapse further limb salvage becomes difficult and many complications may occur.
Adult↗
[High dosage Gd-DPTA-BMA (Gadodiamid) administration in diagnosis and therapeutic monitoring of malignant bone tumors].
PURPOSE: To evaluate the efficacy of high-dose Gd-DTPA-BMA (gadodiamide, Omniscan) as a contrast for magnetic resonance imaging of malignant bone tumors and the use of high-dose dynamic studies for predicting the response to pre-operative chemotherapy. MATERIALS AND METHODS: Examinations were performed in 22 patients with suspected malignant bone tumor on a 1.5 T system. In 8 cases a follow-up examination was done after preoperative chemotherapy. Static studies included Pd- and T2-weighted spin-echo sequences as well as T1-weighted spin-echo sequences, obtained pre- and post-contrast. Dynamic studies were performed using a FLASH 2D-gradient-echo sequence (TR 40 ms/TE 10 ms, 90 degrees flip angle) every 20 s after intravenous bolus injection of Gd-DTPA-BMA (0.3 mmol/kg body weight). MR images were evaluated qualitatively by visual assessment of conspicuity size, extraosseous delineation and structure of the lesion and quantitatively by measurement of the signal intensities and calculation of the relative increase in signal intensity. RESULTS: Qualitative image analysis showed best demonstration of the lesions on contrast-enhanced T1-weighted images. Comparison of T1-weighted pre- and postcontrast spin-echo sequences revealed significantly better assessment of tumor structure after administration of contrast media. After preoperative chemotherapy, all responders showed a markedly stronger reduction in relative increase in signal intensity in dynamic studies compared to nonresponders. CONCLUSION: Gd-DTPA-BMA is effective for magnetic resonance imaging of musculoskeletal lesions and improves assessment of the tumor structure. Dynamic studies may help to predict the response to preoperative chemotherapy.
Adolescent↗
Adjuvant local treatment of parosteal osteosarcoma.
Four patients with parosteal osteosarcoma of the distal femur were treated by resection and insertion of cement. The resection was marginal in 3 patients and intralesional in one. The histology was grade 1 in 3 and grade 2 in one. The cement was subsequently removed and a fibular autograft used for reconstruction. The average follow up was 88 months (range 20 to 161 months). The function of the affected limb was excellent. The best time to remove the cement was after one year.
Adolescent↗
Primary non-Hodgkin lymphomas of the CNS-proliferation, oncoproteins and Epstein-Barr-virus.
Primary cerebral lymphomas (PCL's) are rare tumors which, however, occur with increasing frequency. The present study investigated 55 PCL's of B-cell type, 36 in immunocompetent and 19 in AIDS-patients and 6 cases of intravascular lymphomatosis. In immunocompetent patients, proliferative indices as evaluated by PC10 and MIB1 reflected the histologic grade. Low grade tumors had a mean PCNA and MIB-1 count of 19 and 18.8 (SD 14.7 and 13.2), respectively, and high grade neoplasias showed counts of 56.7 and 47.1 (SD 19 and 17.4), respectively. No correlation of both indices with patient survival was found. 21 cases (58.3%) displayed p53-positivity of varying degree and 19 cases (52.7%) harbored bcl-2 positive neoplastic cells. Immunocompetent cases were always negative for Epstein-Barr virus RNA and lmp-1-protein. In AIDS-cases, 13 cases (68.4%) showed up lmp-1 positivity and 15 cases (78.9%) had EBER-RNA. bcl-2 positive cells were detected in 5 cases (26.3%) and all cases were p53-negative. These results are in keeping with a role of EBV in the pathogenesis of primary cerebral lymphomas in AIDS-, but not in immunocompetent patients. None of the cases with intravascular lymphomatosis showed an expression of bcl-2 or p53 oncoproteins or lmp-1 and none had EBER-RNA.
Adult↗
Intracortical low grade osteosarcoma. A unique case and review of the literature on intracortical osteosarcoma.
The thirteenth case of an intracortical osteosarcoma is presented. According to the authors' knowledge, this case appears to be the first report of an intracortical low grade osteosarcoma of the fibrous dysplasia-like variant in the femur. All other cases (except one small cell variant) reported in the literature were histologically diagnosed as high grade osteoblastic or sclerotic variants. The finding of both a low grade and a small cell variant of osteosarcoma supports the concept that intracortical osteosarcoma rather represents a distinct entity defined by location than an early detected conventional medullary osteosarcoma.
Child↗
Metastasis of chondrosarcoma.
This study was undertaken to analyse metastases of patients with intermediate- or high-grade chondrosarcomas. Out of 24 intermediate-grade tumours, 5 (21%) developed metastases, as did 6 of 10 high-grade tumours (60%) (P = 0.04). Four patients developed pulmonary metastasis only, 5 developed both pulmonary metastases and metastases of the other sites. Two patients showed a rare metastatic pattern: bone metastases only. The metastasis rate in the primary chondrosarcoma (42%) was higher than that in the secondary chondrosarcomas (0%) (P = 0.03). The metastasis rate was higher in patients with local recurrence (86%) than in those without local recurrence (19%) (P = 0.01). In 5 of 6 patients who had a local relapse and metastasis, the interval between the two relapses was a few months.
Adult↗
Metastasis of chondrosarcoma.
This study was undertaken to analyse metastases of patients with intermediate- or high-grade chondrosarcomas. Out of 24 intermediate-grade tumours, 5 (21%) developed metastases, as did 6 of 10 high-grade tumors (60%) (P = 0.04). Four patients developed pulmonary metastasis only, 5 developed both pulmonary metastasis and metastases of the other sites. Two patients showed a rare metastatic pattern: bone metastases only. The metastasis rate in the primary chondrosarcoma (42%) was higher than that in the secondary chondrosarcomas (0%) (P = 0.03). The metastasis rate was higher in patients with local recurrence (86%) than in those without local recurrence (19%) (P = 0.01). In 5 of 6 patients who had a local relapse and metastasis, the interval between the two relapses was a few months.
Cytomegalovirus↗
Wet autoclave pretreatment for immunohistochemical demonstration of oestrogen receptors in routinely processed breast carcinoma tissue.
The immunohistochemical demonstration of oestrogen receptor (OR) was performed on 32 randomly selected and routinely processed breast carcinomas after wet autoclave pretreatment of sections. The autoclave method was compared to the OR status found on frozen sections as well as to alternative pretreatment methods such as enzymatic predigestion and microwave irradiation. Using four different monoclonal antibody clones (H222, LH1, CC4-5, 1D5.26), the OR status was evaluated for each of the various pretreatment methods applied. All cases with a high OR content on frozen sections (n = 11) also showed a high OR status on wet autoclave-pretreated paraffin tissues using antibody clones 1D5.26 and CC4-5; in cases with low OR content on frozen sections, no false-negative cases were recorded using only the antibody 1D5.26 neither after wet autoclave nor microwave pretreatment. In addition, with this antibody, OR was detectable after autoclave pretreatment in two cases which were considered to be OR-negative even on frozen sections. When the primary antibody was omitted, no false-positive cases were observed after wet autoclave pretreatment. Thus, in our hands, wet autoclave pretreatment, in combination with the antibody 1D5.26, offers a highly sensitive method for the immunohistochemical demonstration of OR in routinely formalin-fixed, paraffin-embedded sections of breast carcinomas.
Antibodies, Monoclonal↗
Ewing's sarcoma of the ribs. A report from the cooperative Ewing's sarcoma study.
31 patients with primary Ewing's sarcoma of the ribs were treated according to the protocols of CESS 81, CESS 86P and CESS 86. The results of treatment were reviewed and analysed. 24 patients presented with localised disease and 7 with regional disease. 20 of 24 localised cases and 6 of 7 regional cases underwent tumour resection. All but 2 localised cases received irradiation. The cumulative relapse-free survival (RFS) rate of 31 patients was 61% at 12.8 years. Patients with poor prognosis had tumour of the upper ribs (P = 0.0338), the posterior component of the ribs (P = 0.0597), or regional disease (P = 0.0001). Tumour size, existence of pleural effusion, type of the surgical margin and response to chemotherapy were not significant prognostic factors. Most of the localised cases could be controlled by combined treatment, but in regional cases prognosis remained poor.
Adolescent↗
[Tumorous space-occupying lesions of the pelvic skeleton. A radiological analysis of 234 cases].
PURPOSE: From the material of a bone tumour record file, an attempt was made to determine criteria enabling radiographic prediction of malignancy and tumour entity of lesions of the pelvis. METHODS: Patients' age, location and radiographic morphology of 234 space-occupying lesions of the pelvis were analysed retrospectively. RESULTS: 62.8% of all lesions were malignant, and the portion of malignant tumours increased with increasing age. While 68.0% of the lesions were found in the ilium, 18.8% in the pubis and 13.2% in ischium, the proportion of benign and malignant lesions did not vary in the different bones. Lesions showing a growth rate according to Lodwick grade IA and to IB were benign in 100% and in 82.0%, respectively. In contrast, tumours of grade II or III were malignant in 89.1% and 88.0% of cases, respectively. CONCLUSIONS: By the construction of subgroups by combining the patients' age and growth rate, the prediction of the malignant potential of a lesion increased significantly. The younger the patient, the more aggressively a benign lesion may grow, while the older the patient the slower a malignant tumour may grow. Prediction of the tumour entity is rarely possible.
Adolescent↗
[Tumorous space-occupying lesion of the ilium].
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Biopsy↗
Prognostic features of Ewing's sarcoma on plain radiograph and computed tomography scan after initial treatment.
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Base Sequence↗
Rapid detection of loss of heterozygosity of chromosome 17p by polymerase chain reaction-based variable number of tandem repeat analysis and detection of single-strand conformation polymorphism of intragenic p53 polymorphisms.
Intragenic restriction site polymorphisms in amino acid residue 72 in exon 4 and a Mspl polymorphism in intron 6 of the p53 tumour suppressor gene can both serve as polymorphic markers. Probe YNZ22 (D17S5) is a highly polymorphic, variable number of tandem repeat (VNTR) marker which maps to chromosome 17p13.1 where the p53 gene is located. Locus specific amplification by polymerase chain reaction (PCR) technique and subsequent non-isotopic single-strand conformation polymorphism analysis of the PCR fragments was used for the detection of loss heterozygosity (LOH) of 17p including the p53 gene locus. In combination with a PCR-based method for the analysis of the VNTR locus D17S5 using unique sequences flanking the polymorphic region of YNZ22 we investigated tumour DNA and corresponding constitutional DNA from 69 patients, including 39 patients with gastric cancer, 21 patients with osteosarcomas and 9 patients with Ewing's sarcomas. Using all three methods, 49/69 (71%) patients were informative for LOH, which revealed allelic loss in 5/39 (12.8%) gastric cancers, 1/9 (11.1%) Ewing's sarcoma, and 4/20 (20%) osteosarcomas.
Base Sequence↗