Management of giant desmoid type fibromatosis of abdominal wall

Introduction: Desmoid type fibromatosis (DT) represents a rare benign tumor formation characterized by local aggressive behavior but an absence of metastatic potential. Histologically, they are often misdiagnosed, leading to unnecessary radical surgery. Even with proper diagnosis, there are different approaches and unclear protocols for the treatment of DT. Case Presentation: The study presents the case of a 33-year-old male who presented to the emergency department with a large swelling of the abdominal wall that had been present for two years. An ultrasound was performed, and the abdominal swelling was described by the radiologist as a ventral hernia. After further radiological studies (CT scan and magnetic resonance imaging), a diagnosis of a tumor of the abdominal wall was established. A partial biopsy led to the diagnosis of sarcoma. Surgery was planned as the tumor was growing rapidly, reaching around 30 cm and causing necrosis of the abdominal wall skin. After the resection of 8.5 kg of tumor with clinically clear margins, the defect was reconstructed by placing a polypropylene mesh and using a darn nylon suture with a local rotational skin flap. In the postoperative days, an infection of the surgical wound developed. The infection resolved in two weeks without further complications. The definitive histopathological diagnosis was desmoid type fibromatosis. Conclusion: Active surveillance is now regarded as the most acceptable strategy for the treatment of DT. A better understanding of the genetic alterations underlying DT and the identification of prognostic factors that favor aggressive behavior will lead to the development of appropriate individual strategies and tailored therapy for each patient.