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Updated: Aug 20, 2026

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
[Desmoid-type fibromatosis (aggressive fibromatosis)]
C Kuhnen1, M Helwing, S Rabstein
1Institut für Pathologie, Register für Gliedmassentumoren, Berufsgenossenschaftliche Kliniken Bergmannsheil, Ruhr-Universität Bochum. Cornelius.Kuhnen@ruhr-uni-bochum.de
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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