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  • 1
    ISSN: 1432-1084
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Histopathology 41 (2002), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aim:  Clear cell sarcoma is a high-grade sarcoma with morphological features resembling malignant melanoma. This tumour is reported to display a characteristic distribution pattern nearly always involving the extremities. We report the first case of clear cell sarcoma of the stomach.Methods and results:  A 30-year-old male developed a huge tumour of the stomach, which at first glance could be considered as a poorly differentiated carcinoma. Immunohistochemical and ultrastructural examinations were consistent with a diagnosis of metastatic melanoma. However, cytogenetic examination revealed a t(12;22) translocation, specific for clear cell sarcoma. This was confirmed by fluorescence in-situ hybridization.Conclusion:  Making a reliable diagnosis of clear cell sarcoma of the stomach requires cytogenetic or molecular diagnostic investigations, particularly to rule out metastatic melanoma. This diagnosis avoids an unnecessary search for a primary melanoma.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European radiology 10 (2000), S. 207-212 
    ISSN: 1432-1084
    Keywords: Key words: Bone neoplasms/di diagnosis – Bone neoplasms/dt therapy – Neoplasm recurrence – Local/di diagnosis – Neoplasm staging – Magnetic resonance imaging – Ultrasound – CT
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We present an overview of the imaging modalities in bone tumors. The imaging strategies of preoperative work-up, monitoring the effect of chemotherapy and the detection of recurrences by long-term follow-up are discussed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Der Radiologe 38 (1998), S. 502-508 
    ISSN: 1432-2102
    Keywords: Schlüsselwörter Chondrosarkom ; Radiologische Diagnose ; Magnetresonanztomographie (MRI) ; Kontrastmittel ; Key words Cartilage ; Sarcoma ; Diagnostic radiology ; Magnetic resonance (MR) ; contrast agents
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Malignant cartilaginous tumors (chondrosarcomas) are, with a relative frequency of 20%, the second most common malignant tumors of bone after osteosarcoma. The diagnosis of chondrosarcoma can usually be made confidently based on combination of clinical information, radiographs, Gd-enhanced MR imaging, and histologic examination of a biopsy sample. The combination of these parameters is important because accuracy of histologic diagnosis is adversely affected by unrepresentative sampling of these usually large tumors. The prognosis of patients with chondrosarcoma becomes poorer with more axial location, higher histologic grade, larger tumor size and inadeqaute resection. By careful analysis of radiographs and Gd-enhanced MR imaging the radiologist has the ability to improve the management of patients with chondrosarcoma.
    Notes: Zusammenfassung Die malignen Knorpeltumoren oder Chondrosarkome treten in einer Inzidenz von 20% auf und sind damit die zweithäufigsten primären malignen Tumoren des Knochens nach dem Osteosarkom. Üblicherweise kann in Zusammenschau von klinischer Information, Nativröntgenbildern und Kontrastmittel-MR-Untersuchung sowie Histologie eine zuverlässige Diagnose gestellt werden. Es ist wichtig, alle diese Modalitäten in ihrer Gesamtheit zu betrachten, da die Histologien manchmal durch nichtrepräsentative Gewebsentnahmen aus diesen oft großen Tumoren ungenau sind. Die Prognose von Chondrosarkomen verschlechtert sich bei stammnaher Lokalisation, hohem Grading, zunehmender Tumorgröße und insuffizienter Resektion. Durch sorgfältige Interpretation von Röntgenaufnahmen und MR-Kontrastmitteluntersuchungen liegt es in der Hand des Radiologen die Behandlung von Chondrosarkompatienten zu optimieren.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-2161
    Keywords: Bone tumour ; Bone neoplasm ; Ewing's sarcoma ; MR imaging ; Chemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Magnetic resonance (MR) imaging was performed in 26 patients with Ewing's sarcoma of bone preceding and following neoadjuvant chemotherapy, to assess tumour response non-invasively prior to surgery. T1- and T2-weighted spin echo images were obtained. Changes including intra- and extramedullary signal intensities, tumour demarcation, tumour volume and the appearance of residual extramedullary tumour were compared with histopathology of the resected specimens. Reduction of tumour volume was significantly higher in good responders. Other single parameters did not correlate with histologic tumour response. However, when several MR parameters summarized in a classification system were combined, a positive correlation with histopathologic response was found. A limited decrease of tumour volume (〈25%) and/or residual soft tissue mass following chemotherapy correlated with a poor response. An inhomogeneous, well-defined cuff of abnormal tissue encircling the bone and/or radiological disappearance of the soft tissue tumour component following chemotherapy correlated with good response. Twenty-three out of 26 patients were correctly classified by MR as good or poor responders. Minimal residual disease (〈10% of the entire tumour volume), observed histologically, could not be identified with MR imaging. Tumour volume reduction and residual extramedullary tumour, rather than changes of signal intensity, are major features for evaluating the response to chemotherapy in Ewing's sarcoma.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-2161
    Keywords: Bone tumour ; Bone neoplasm ; Ewing's sarcoma ; Chemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A uniform classification of response to chemotherapy is essential to allow comparison of local effect and ultimate prognosis between different therapy schedules. We define a histological grading system for assessment of the response to chemotherapy in Ewing's sarcoma, based on the amount and architectural pattern of residual histologically viable-appearing tumour, the preferential sites of minimal residual tumour and the amount of tumour necrosis. Twenty-six consecutive patients with a biopsy-proven Ewing's sarcoma were treated with chemotherapy prior to surgery. The effect of chemotherapy was evaluated microscopically on the specimens obtained after surgery. Response to chemotherapy was classified as minimal or no effect (〈10% tumour necrosis), moderate effect (solid areas of remnant viable tumour), minimal residual disease, and no evidence of disease (100% tumour necrosis or well-vascularized fibrous tissue). The subperiosteal area in particular, and, less frequently, soft tissues and intramedullary compartment were identified as sites of predilection for persistence of microscopic viable tumour foci, frequently depicted as pseudo-rosettes in a characteristic scattered pattern. Although it is not well known whether morphological viability of these residual clusters in Ewing's sarcoma indicates biological viability, accurate preoperative local staging, with special attention to preferential sites of residual viable tumour, is essential. The proposed grading system can be used to standardize assessment of chemotherapy in trials, and may serve as a standard for non-invasive monitoring of preoperative chemotherapy with magnetic resonance imaging.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-2161
    Keywords: Key words Bone tumour ; Bone neoplasm ; Conventional radiography ; Chemotherapy ; Osteosarcoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Objective. The objective of this study was to assess the effectiveness of conventional radiography in predicting histopathologic response in patients with osteogenic sarcoma who were treated with preoperative chemotherapy. Design and patients. The radiographs of 22 patients with an osteogenic sarcoma, taken before and after neoadjuvant chemotherapy, were reviewed. Tumour location, size, radiographic appearance, margination, cortical destruction and periosteal reaction were evaluated. The findings were correlated with the histopathologic response of the surgical specimen. Results. None of the findings proved to be of predictive value for the histopathologic response. Increase in tumour diameter and increase in ossification and/or calcification, which were seen in more than half of the patients, did not correlate with response. Conclusion. Conventional radiographs do not contribute to the identification of good or poor responders.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-2161
    Keywords: Key words Osteosarcoma ; Distal femur ; MR imaging ; Limb salvage surgery ; Surgical planning
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Objective. To analyse to what extent MR imaging based decisions were correct in determining the surgical procedure in patients with osteosarcoma of the distal femur. Design. We compared the findings on MR imaging that determined the surgical strategy with the actual surgical findings or histopathological specimen. We assessed to what extent MR images could be used to determine the surgical procedure in patients with osteosarcoma. Patients. Between 1985 and 1992, 34 patients with an osteosarcoma were treated. Two patients had a low-grade osteosarcoma. Thirty-two patients with a high-grade osteosarcoma of the distal femur (17 stage IIB, 15 stage III) were included in this study. Surgical options consisted of either limb salvage surgery or ablative surgery, which included the Van Nes-Borggreve rotationplasty. Limb salvage surgery consisted of extra-articular or transarticular resection, followed by reconstruction. Surgery was planned depending on the local extent of the tumour as depicted on the MR studies, coupled with data from the biopsy, age, patient compliance and histological grade. Follow-up was available in all patients. Nine of 17 patients graded as IIB were alive with an average follow-up of 68 months (range 46–109 months), of whom one has metastases. No local recurrence was encountered. Results. If no tumour involvement on MR imaging was found and this was used as a determining factor, this proved to be correct at subsequent analysis. We found that in eight of 11 cases, when MR images suggested a close relationship between tumour and nerve, an oncologically safe plane could be achieved during surgery. In three, a free plane could not be accomplished, as confirmed at histopathological examination. Hence, when nerve involvement was equivocal on MR imaging we found it valuable to reassess nerve involvement during surgery and reconsider limb salvage surgery. When the decisive factor was the vascular involvement and tumour extension was read as equivocal, it was possible to obtain a oncologically safe plane in six of 13 cases. When comprehensive tumour involvement of any structure was noted pre-operatively, it proved to be correct at histopathological examination, except for one case of assumed vascular involvement that proved to be free. Conclusions. If no tumour involvement of a structure was found on MR imaging and this was used as a determining factor, this proved to be correct. When nerve involvement was equivocal on MR imaging we found it valuable to reassess nerve involvement during surgery and reconsider limb salvage surgery. Extensive tumour involvement of any structure, as shown by MR imaging, could be used correctly as a decisive argument in planning a surgical procedure.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-2161
    Keywords: Key words Clear cell sarcoma ; Malignant melanoma of soft parts ; MRI ; Magnetic resonance ; Neoplasm
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Objective. To evaluate MR imaging and pathology findings in order to define the characteristic features of clear cell sarcoma of the soft tissues (malignant melanoma of the soft parts). Design and patients. MR examinations of 21 patients with histologically proven clear cell sarcoma of the musculoskeletal system were retrospectively reviewed and assessed for shape, homogeneity, delineation, signal intensities on T1- and T2-weighted images, contrast enhancement, relationship with adjacent fascia or tendon, secondary bone involvement, and intratumoral necrosis. In 19 cases the pathology findings were available for review and for a comparative MR-pathology study. Results. On T1-weighted images, lesions were isointense (n=3), hypointense (n=7) or slightly hyperintense to muscle (n=11). Immunohistochemical examination was performed in 17 patients. All 17 specimens showed positivity for HMB-45 antibody. In nine of 11 lesions with slightly increased signal intensity on T1-weighted images, a correlative MR imaging-pathology study was possible. All nine were positive to HMB-45 antibody. Conclusions. Clear cell sarcoma of the musculoskeletal system often has a benign-looking appearance on MR images. In up to 52% of patients, this lesion with melanocytic differentiation has slightly increased signal intensity on T1-weighted images compared with muscle. As the presence of this relative higher signal intensity on T1-weighted images is rather specific for tumors displaying melanocytic differentiation, radiologists should familiarize themselves with this rare entity and include it in their differential diagnosis when confronted with a well-defined, homogeneous, strongly enhancing mass with slightly higher signal intensity compared with muscle on native T1-weighted images.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1569-8041
    Keywords: chemotherapy ; osteosarcoma ; relative dose intensity ; survival ; tumour response
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background: Despite advances in the treatment of primary limb osteosarcoma, the outcome of patients with primary metastatic and axial skeletal disease remains poor. The European Osteosarcoma Intergroup have assessed a combination chemotherapy regimen consisting of ifosfamide (IFOS) 3 g/m2/d1–2, doxorubicin (DOX) 25 mg/m2/d1–3 i.v. bolus and cisplatin (CDDP) 100 mg/m2/d1. Patients and methods: One hundred nine previously untreated patients with primary osteosarcoma were registered. Eligibility was confirmed in 103. At presentation, 45 eligible patients had metastatic disease, 15 axial skeletal primary tumours and 43 non-metastatic limb tumours. Results: The major toxicities were myelosuppression (90%, grade 3 or 4) and nausea and vomiting (74%, grade 3 or 4). Overall mean relative dose intensity (RDI) was 80% (88% CDDP, 75% IFOS, 81% DOX). Clinical response as measured by reduction in tumour volume occurred in 36% (95% confidence interval (95% CI): 27%–47%) of primary tumours. Response of pulmonary metastases to chemotherapy was seen in 33% (95% CI: 19%–49%). Good histological response (≥90% necrosis of the tumour) occurred in 33% (95% CI: 22%–45%) of resected tumours. Five-year survival was 62% in limb-non-metastatic, 41% in axial skeletal and 16% in limb metastatic patients. Conclusions: This regimen is active in osteosarcoma but does not appear to be more active than the two-drug CDDP–DOX regimen currently recommended by EOI.
    Type of Medium: Electronic Resource
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