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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
    ISSN: 1432-0584
    Keywords: Acute myelogenous leukemia ; Allogeneic marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Between February 1982 and April 1995, 62 patients (37 male, 25 female) with acute myelogenous leukemia (AML) with a median age of 32 years (19–51 years) received allogeneic marrow grafts from an HLA-identical sibling (n=60) or an HLA-mismatched family member (n=2). At the time of transplant, 35 patients were in first complete remission (CR), five in second CR, eight were primary refractory, eight were in untreated relapse and six in refractory relapse. The FAB subtypes were as follows: M1 (n=17), M2 (n=13), M3 (n=6), M4 (n=19), M5 (n=6), M6 (n=1). For conditioning most patients were given total body irradiation combined with cyclophosphamide (CY,n=50) or CY and busulfan (n=9). For graft-versus-host disease prophylaxis patients received cyclosporin A (CSA) and methotrexate (MTX) (n=32), MTX alone (n=12), CSA and methylprednisone (n=5), or CSA alone (n=13). As of April 1995, probability of leukemia-free survival projected at 10 years after BMT was 60% for patients transplanted in first CR compared with 10% for patients transplanted beyond first CR. Transplant-related mortality was 11% after BMT in first CR and 39% after BMT beyond first CR. Probability of relapse projected at 10 years after BMT is 32% for patients who received transplants in first CR and 81% for patients who received transplants beyond first CR. Thus, high-dose chemo/radiotherapy followed by allogeneic marrow infusion has a high curative potential for patients with AML who receive transplants in first CR and offers the chance of long-term disease-free survival for some patients with advanced disease.
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  • 3
    ISSN: 1432-0584
    Keywords: Key words Acute myelogenous leukemia ; Allogeneic marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Between February 1982 and April 1995, 62 patients (37 male, 25 female) with acute myelogenous leukemia (AML) with a median age of 32 years (19–51 years) received allogeneic marrow grafts from an HLA-identical sibling (n=60) or an HLA-mismatched family member (n=2). At the time of transplant, 35 patients were in first complete remission (CR), five in second CR, eight were primary refractory, eight were in untreated relapse and six in refractory relapse. The FAB subtypes were as follows: M1 (n=17), M2 (n=13), M3 (n=6), M4 (n=19), M5 (n=6), M6 (n=1). For conditioning most patients were given total body irradiation combined with cyclophosphamide (CY, n=50) or CY and busulfan (n=9). For graft-versus-host disease prophylaxis patients received cyclosporin A (CSA) and methotrexate (MTX) (n=32), MTX alone (n=12), CSA and methylprednisone (n=5), or CSA alone (n=13). As of April 1995, probability of leukemia-free survival projected at 10 years after BMT was 60% for patients transplanted in first CR compared with 10% for patients transplanted beyond first CR. Transplant-related mortality was 11% after BMT in first CR and 39% after BMT beyond first CR. Probability of relapse projected at 10 years after BMT is 32% for patients who received transplants in first CR and 81% for patients who received transplants beyond first CR. Thus, high-dose chemo/radiotherapy followed by allogeneic marrow infusion has a high curative potential for patients with AML who receive transplants in first CR and offers the chance of long-term disease-free survival for some patients with advanced disease.
    Type of Medium: Electronic Resource
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