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  • Alveolar soft part sarcoma  (1)
  • Keywords: Renal cell carcinoma; stereotactic radiosurgery; metastasis; Gamma Knife  (1)
  • 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.
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  • 2
    ISSN: 0942-0940
    Keywords: Keywords: Renal cell carcinoma; stereotactic radiosurgery; metastasis; Gamma Knife
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary  From Jan. 1993 to Sept. 1995 23 patients suffering from brain metastases from renal cell carcinoma were treated with the Leksell Gamma Knife at the University of Vienna. At the time of diagnosis 13 patients had single and 10 patients presented with multiple metastatic lesions with a total of 44 metastases in MRI scans. Median tumour volume was 5500 cmm (range 100–24000 cmm). Predominant neurological symptoms and signs were different forms of hemiparesis, focal and generalized seizures, cognitive deficit, headache, dizziness, ataxia and CN XII paresis.  Fourteen patients received Gamma Knife Radiosurgery (GKRS) with a median dose of 22 Gy (range 8–30 Gy) at the tumour margin. Nine patients underwent a combined treatment of a radiosurgical boost with a median dose of 18 Gy (range 10–22 Gy) at the tumour margin followed by Whole Brain Radiotherapy (total dose 30 Gy/2 weeks).  In 20 patients tumour volume reduction up to 30% of the primary tumour volume was found after 4 weeks, evaluated on CT or MRI. A total remission was seen in 4 cases 3 months after GKRS. We achieved a local tumour control of 96%. Rapid neurological improvement after GKRS was seen in 17 patients. The median survival time was 11 months; the one-year actual survival in this unselected group was 48%. Five long term survivors were still alive, 18 patients had subsequently died, 15 of them of general tumour progression.  GKRS induces a significant tumour remission accompanied by rapid neurological improvement and therefore provides the opportunity for extended high quality survival. Neither local tumour control was improved nor CNS relapse free survival was prolonged significantly by additional WBRT.
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