Summary
The leiomyosarcoma of vascular origin is a very rare tumor which is rarely recovered before the disease has reached an advanced stage because of nonspecific and late symptoms. This is a report of 3 cases with leiomyosarcoma of the vena cava and a review of the literature. A high local recurrence rate and high frequency of distant metastases are responsible for the bad long-term prognosis. Early hematogenous tumor spread is favored by intraluminal tumor growth. Only complete surgical resection - if necessary with vascular reconstruction — can improve the bad long-term prognosis.
Zusammenfassung
Das Leiomyosarkom vaskulären Ursprungs ist ein äußerst seltener Tumor, der aufgrund seines symptomarmen Verlaufs meist erst in weit fortgeschrittenem Stadium entdeckt wird — dann mit sehr schlechter Prognose. Anhand dreier Kasuistiken mit V. cava Leiomyosarkomen (LMS) werden Diagnostik, Therapie and Verlauf beschrieben and anhand einer ausführlichen Literaturübersicht diskutiert. Neben der hohen Lokalrezidivrate sind Fernmetastasen für die Langzeitprognose entscheidend. Bei den Vena cava — LMS ist eine frühe hämatogene Tumorpropagation durch Abschwemmen intraluminaler Tumoranteile wahrscheinlich und für die schlechte Prognose verantwortlich. Nur die frühzeitige komplette Tumorresektion, gegebenenfalls mit Gefäßersatz, kann die Prognose des V. cava-Leiomyosarkoms verbessern.
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Sturm, J., Raute, M. & Trede, M. Das Leiomyosarkom der V. cava Fallbeschreibung und Literaturübersicht. Langenbecks Arch Chir 376, 182–188 (1991). https://doi.org/10.1007/BF00250345
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DOI: https://doi.org/10.1007/BF00250345