Configuration of Primary and Recurrent Aggressive Fibromatosis on Contrast-Enhanced MRI with an Evaluation of Potential Risk Factors for Recurrences in MRI Follow-Up

Abstract

Purpose To analyze the appearance of primary and recurrent aggressive fibromatosis (AF) on MRI with a focus on configuration and to determine potential risk factors for recurrences detected on MRI follow-up scans.

Methods From 79 consecutive patients with histologically proven diagnosis of AF, 39 patients underwent a minimum of four 1.5 T MRI follow-up scans after resection of primary AF between 2008 and 2018. The primary and recurrent tumors were radiographically examined for configuration, limitation and extent on MRI. Epidemiological data and loco-regional subcutaneous edema, muscle edema and post-operative seroma were included.

Results The mean age of the patients was 39 ± 2.6 years. Primary and recurrent AF most often occurred in the thigh. The main appearance of primary AF was significantly most often fascicular (p = 0.001–0.01) with heterogeneous and marked contrast enhancement. 21 % (n = 8) of the patients developed recurrences of AF. A fascicular configuration with homogeneous/heterogeneous contrast enhancement was the main appearance of recurrent AF, but recurrent AF appeared nodular, polycyclic, ovoid or streaky/flat as well. Recurrent AF significantly most often occurred within the first 9 months after primary tumor resection (p = 0.009), especially in patients up to 25 years of age (RR = 6.1; 95 % CI: 1.8–20.9; p = 0.004). The cases of recurrent AF were altogether significantly smaller than the primary tumors (p = 0.001). Post-treatment subcutaneous and muscle edema were present in 77 % and 56 %, respectively. Patients with muscle edema after primary tumor resection had a significantly higher risk for AF recurrences (relative risk ratio (RR) = 1.8; 95 % CI: 1.16–2.8; p = 0.0096). There was no significant difference detected in patients with complete or incomplete resection of the primary tumor.

Conclusion Primary and recurrent aggressive fibromatosis has a mostly fascicular configuration, but may appear ovoid, nodular, streaky/flat or polycyclic as well. High risks for tumor recurrences are detected for patients up to 25 years of age, patients within the first 9 post-operative months and patients with muscle edema after primary tumor resection.

Key points: 

• Primary aggressive fibromatosis mostly has a fascicular configuration with heterogeneous contrast enhancement

• Recurrent aggressive fibromatosis usually has a fascicular configuration with heterogeneous/homogeneous contrast enhancement

• Patients within the first 9 post-operative months and up to 25 years of age have a significantly higher risk for recurrences

• Muscle edema after resection of primary aggressive fibromatosis is associated with a significantly higher risk for recurrences

Citation Format

• Sedaghat S, Surov A, Krohn S et al. Configuration of Primary and Recurrent Aggressive Fibromatosis on Contrast-Enhanced MRI with an Evaluation of Potential Risk Factors for Recurrences in MRI Follow-Up. Fortschr Röntgenstr 2020; 192: 448 – 457

Zusammenfassung

Zielsetzung Analyse der Erscheinung primärer und rezidivierender aggressiver Fibromatosen (AF) in der MRT mit Fokus auf Tumorkonfiguration und Evaluation potenzieller Risikofaktoren für Rezidive in MRT-Verlaufskontrollen.

Methode Von 79 Patienten mit histologisch gesicherter Diagnose „aggressive Fibromatose“ unterzogen sich zwischen 2008 und 2018 39 Patienten mindestens 4 1,5 T-MRT-Verlaufskontrollen nach Resektion der Primarien. Die Primär- und Rezidivtumoren wurden radiografisch auf Konfiguration, Begrenzung und Ausdehnung in der MRT untersucht. Epidemiologische Daten und lokoregionäre Weichgewebsveränderungen (subkutane Ödeme, Muskelödeme und postoperative Serome) wurden eingeschlossen.

Ergebnisse Das mittlere Alter der Patienten betrug 39 ± 2,6 Jahre. Primäre und rezidivierende AF traten bevorzugt am Oberschenkel auf. Primäre AF waren signifikant häufig faszikulär konfiguriert (p = 0,001–0,01) und wiesen eine inhomogene und kräftige Kontrastmittelaufnahme auf. Bei 21 % der Patienten (n = 8) fanden sich im Verlauf Tumorrezidive. Die Tumorrezidive waren meist ebenfalls faszikulär konfiguriert und wiesen ein homogenes/inhomogenes und kräftiges Kontrastmittelenhancement auf. Dennoch können Rezidive aggressiver Fibromatosen auch nodulär, polyzyklisch, oval oder streifig konfiguriert sein. Rezidive aggressiver Fibromatosen traten signifikant häufig in den ersten 9 postoperativen Monaten (p = 0,009) und insbesondere bei Patienten bis 25 Jahre auf (relatives Risiko (RR) = 6,1; 95 %-KI: 1,8–20,9; p = 0,004). AF-Rezidive waren im Vergleich signifikant kleiner als Primärtumoren (p = 0,001). Posttherapeutische subkutane und muskuläre Ödeme zeigten sich bei 77 % bzw. 56 % der Patienten. Patienten, die nach Primärtumorresektion Muskelödeme aufwiesen, zeigten ein signifikant erhöhtes Risiko für Rezidive (RR = 1,8; 95 %-KI: 1,16–2,8; p = 0,0096). Es ergab sich kein signifikanter Unterschied zwischen vollständiger und inkompletter Resektion in Bezug auf die Rezidivrate.

Schlussfolgerung Primäre und rezidivierende aggressive Fibromatosen weisen meist eine faszikuläre Konfiguration auf. Dennoch können die Tumoren auch oval, nodulär, streifig oder polyzyklisch konfiguriert sein. Ein erhöhtes Risiko besteht für Patienten bis 25 Jahre, innerhalb der ersten 9 postoperativen Monate und bei Vorhandensein von Muskelödemen nach primärer Tumorresektion.

Kernaussagen:

• Primäre aggressive Fibromatosen sind meist faszikulär konfiguriert und weisen eine inhomogene Kontrastmittelaufnahme auf

• Rezidivierende aggressive Fibromatosen sind in der Regel ebenfalls faszikulär konfiguriert und zeigen eine inhomogene oder homogene Kontrastmittelaufnahme

• Patienten innerhalb der ersten 9 postoperativen Monate und bis 25 Jahre weisen ein signifikant erhöhtes Risiko für Rezidive auf

• Muskelödeme nach Resektion einer primären aggressiven Fibromatose sind assoziiert mit einem signifikant höheren Risiko für Rezidive

Key words

aggressive fibromatosis - configuration - MRI - risk factors - recurrence - post-treatment

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