Zusammenfassung
Desmoidfibromatosen (Synonym: aggressive Fibromatosen) stellen lokal aggressiv wachsende Weichgewebsneoplasien mit hoher Lokalrezidivrate dar. Desmoidtumoren von 33 Patienten im Erwachsenenalter wurden nach klinischen und morphologischen Parametern analysiert (Geschlechts-, Altersverteilung, Lokalisation, Tumorgröße, Mitoserate, Tumormikrogefäßdichte, Resektionsstatus, adjuvante Strahlentherapie). Mögliche statistische Korrelationen wurden mittels Log-rank-Testverfahren überprüft. Eine eindeutige prognostische Wertigkeit der Tumormikrogefäßdichte war nicht abzuleiten. Eine Korrelation zwischen mitotischer Aktivität (eine oder mehr Mitosen/50 HPF) und Lokalrezidivrate konnte nachgewiesen werden, jedoch ohne statistische Signifikanz (log-rank: 0,17). Für die postoperative Radiotherapie resultierte eine statistisch signifikante Bedeutung zur Vermeidung von Lokalrezidiven (log-rank: 0,01). Die mitotische Aktivität bei aggressiven Fibromatosen kann als Hinweis auf ein erhöhtes Potenzial für die Entwicklung eines Lokalrezidivs gewertet werden. Eine postoperative Strahlentherapie erscheint daher sinnvoll. Wesentliche Differenzialdiagnosen der Desmoidfibromatose im Erwachsenenalter umfassen weitere fibroblastisch bzw. myofibroblatisch differenzierte Weichgewebstumoren wie z. B. die Fasziitis nodularis, das Fibrosarkom und niedrig-maligne fibromyxoide Sarkom, das myofibroblastische Sarkom sowie das Leiomyosarkom und Leiomyom des tiefen Weichgewebes.
Abstract
Desmoid-type fibromatoses (aggressive fibromatoses) represent infiltrative, locally destructively growing soft tissue tumors with a high potential for recurrence. Desmoid tumors of 33 adult patients were analysed regarding clinical and morphological aspects (sex, age distribution, site, size, mitotic rate, tumor microvessel density, surgical margins, additional radiotherapy). Possible statistical correlations were examined using log-rank-tests. No prognostic significance of tumor microvessel density was evident. A correlation between mitotic index (1 or more mitoses per 50 high power fields) and local relapse rate was notably striking, but not statistically significant (log-rank: 0.17). Additional postoperatively applied radiotherapy proved to be statistically significant to avoid local recurrences (log-rank: 0.01). The presented results may indicate an increased risk for local relapse in those desmoid-type fibromatoses which are mitotically active. Postoperative radiotherapy seems to be effective in the treatment of aggressive fibromatosis to avoid tumor recurrence. Differential diagnosis of desmoid-type fibromatosis/aggressive fibromatosis in adulthood include various fibroblastic/myofibroblastic soft tissue tumors such as nodular fasciitis, fibrosarcoma, low-grade fibromyxoid sarcoma, myofibroblastic sarcoma as well as leiomyosarcoma and soft tissue leiomyoma.
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Die Autoren danken Frau Cornelia Troske für das Fotodesign.
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Kuhnen, C., Helwing, M., Rabstein, S. et al. Desmoidfibromatosen (aggressive Fibromatosen). Pathologe 26, 117–126 (2005). https://doi.org/10.1007/s00292-004-0742-1
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DOI: https://doi.org/10.1007/s00292-004-0742-1