Sporadic Retroperitoneal Aggressive Fibromatosis: Report of a Case

Abstract

We describe herein a case of sporadic retroperitoneal aggressive fibromatosis. A 54-year-old man with a palpable abdominal mass was referred to our hospital for investigation and treatment. Abdominal ultrasonography and computed tomography revealed a solid mass with relatively well-defined borders in the left abdominal retroperitoneum. At surgery, a large tumor (14 × 13 × 11 cm) was found, arising from the retroperitoneal space and involving the wall of jejunum. Complete removal was achieved. Histological examination of the resected specimen revealed the presence of changes consistent with aggressive fibromatosis. The patient was not scheduled for any further treatment. Two years after surgery, the patient is without any signs of recurrent disease. Although extremely rare, aggressive fibromatosis should be considered in the differential diagnosis of retroperitoneal masses. Complete surgical resection with negative pathological margins remains the first line management of these neoplasms. Careful follow-up is indicated because recurrence may occur every time after surgery. 

Zusammenfassung

Wir beschreiben einen Fall von sporadischer retroperitonealer aggressiver Fibromatose. Ein 54-jähriger Mann wurde uns mit einem tastbaren Abdominaltumor zur Abklärung und Behandlung zugewiesen. Bei der Ultraschalluntersuchung und im CT fand sich eine solide Masse mit relativ gut definierten Grenzen im linken Retroperitoneum. Bei der Operation zeigte sich ein großer Tumor (14 × 13 × 11 cm), der vom Retroperitoneum ausging und die Jejunalwand infiltriert hatte. Der ­Tumor wurde komplett entfernt. Die histologische Untersuchung ergab Veränderungen, die mit einer aggressiven Fibromatose vereinbar waren. Eine weitere Therapie wurde nicht ange­schlossen. Zwei Jahre nach der Operation ist der Patient ohne Anzeichen für ein Rezidiv. Obwohl extrem selten sollte die aggressive Fibromatose in die Differenzialdiagnose eines retroperitonealen Tumors eingeschlossen werden. Therapie der Wahl ist die komplette chirurgische Resektion mit negativen pathologischen Grenzen (R 0-Resektion). Eine sorgfältige Nachbeobachtung ist angezeigt, da diese Tumoren jederzeit nach dem Eingriff zum Rezidiv neigen. 

retroperitoneal fibromatosis - desmoid tumor - surgery

retroperitoneale Fibromatose - Desmoidtumor - Chirurgie

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