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  • 1
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Subkutane Weichteilknoten ; Differentialdiagnose ; Alveolar soft part sarcoma ; Therapie ; Key words Subcutaneous mass ; Differential diagnosis ; Alveolar soft part sarcoma ; Treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: A slow growing indolent subcutaneous mass can be caused by a variety of benign and malignant soft tissue proliferations, however, it is often the first sign of soft tissue sarcomas. Radiological findings are unspecific and biopsy with histopathological analysis is needed. Method: Three female patients (age 9, 191/12 and 232/12 years) are presented with a slow growing subcutaneous mass, which was shown to be a malignant non-embryonal soft tissue sarcoma (alveolar soft part sarcoma). Results: Alveolar soft part sarcoma (ASPS) is a very rare malignancy. At diagnosis, 2 patients already had metastatic disease. One girl died before therapy, the other one suffers from slowly progressive pulmonary metastases 41/2 years after diagnosis, despite multi-modal treatment approaches. The third patient received polychemotherapy and local irradiation after complete tumor resection and is free of disease 8 months after diagnosis. Conclusion: ASPS is a rare slow growing malignancy with an extraordinary tendency to metastasize. The prognosis depends on wether the tumor is operable and whether there are metastases.
    Notes: Zusammenfassung Fragestellung: Langsam wachsende nicht schmerzhafte subkutane Knoten können durch unterschiedlichste gut- oder bösartige mesenchymale Proliferationen verursacht sein. Da sich hinter diesem Zeichen oft ein Malignom verbirgt und bildgebende Analysen nur selten den Weg zur Diagnose weisen, ist eine bioptische Klärung dringend erforderlich. Methode: Wir berichten über 3 Patientinnen (Alter 9, 191/12 und 231/6 Jahre) bei denen sich hinter einem langsam wachsenden subkutanen Knoten ein nicht embryonales Sarkom (Alveolar soft part sarcoma) verbarg. Ergebnisse: Das Alveolar soft part sarcoma (ASPS) ist ein ausgesprochenes seltenes Malignom. Zum Diagnosezeitpunkt lag bei 2 Patientinnen bereits eine Metastasierung vor. Eine Patientin verstarb noch vor Behandlungsbeginn. Bei der 2. sind Lungenmetastasen trotz multimodaler Therapie 41/2 Jahre nach Diagnosestellung langsam progredient. Die 3. Patientin erhielt nach Resektion eines lokalisierten ASPS am Oberarm postoperativ eine Polychemo- und lokale Strahlentherapie und ist 8 Monate nach der Diagnosestellung ohne Krankheitszeichen. Schlußfolgerung: Das ASPS ist ein langsam wachsender Tumor mit ausgeprägter Metastasierungstendenz. Die Prognose ist im wesentlichen von der Operabilität des Primärtumors und dem Vorliegen von Metastasen abhängig.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 66 (1988), S. 337-339 
    ISSN: 1432-1440
    Keywords: Seminoma ; HLA-association ; Genetic factors ; Pathogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical and epidemiological studies suggest that genetic factors may be involved in the etiology and pathogenesis of testicular germ-cell tumors (GCTs). Previous HLA studies have tried to support the concept of genetic anticipation of GCTs, however, the results obtained have been inconsistent. The reasons for the divergent results are mainly statistical problems, i.e., small patient populations, high numbers of antigens tested, and inhomogeneous study populations. In the present retrospective study, 52 patients with histologically pure seminoma were typed for their HLA-A, B, C, and DR antigens. Only BW41 proved to be significantly increased in frequency after correction for the number of antigens tested (chiQ=12.73;P=0.0005). HLA-DR1 was shown to be decreased, however, the difference was not significant. Regarding metastatic seminoma alone, there was a trend towards an increase of A29, BW41, and DR7. Our study gives only weak evidence for the participation of genetic factors in the pathogenesis of seminoma. The statistical trend of HLA association observed in metastatic seminoma deserves further concern as does the question of whether MHC class II antigens are also involved in the pathogenesis of testicular seminoma.
    Type of Medium: Electronic Resource
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