Extra-Abdominal Desmoid Tumours: A Review of the Literature

Abstract

Extra-abdominal desmoid lesions, otherwise known as aggressive fibromatosis, are slow-growing benign lesions which may be encountered in clinical practice. Recent controversies exist regarding their optimal treatment. Given their benign nature, is major debulking surgery justified, or is it worth administering chemotherapy for a disease process which unusually defies common teaching and responds to such medications? We present a literature review of this particular pathology discussing the aetiology, clinical presentation, and various current controversies in the treatment options.

Desmoid Tumor Causing Hip Joint Contracture: A Case Report

“…The majority of patients present with painless swelling. CT is of limited value given the similar attenuation between muscle and extra-abdominal desmoid lesions, as illustrated in the current study [6]. MRI is the modality of choice to assess both the size of the mass and its intimate association with surrounding structures.…”

“…With an increased knowledge of these lesions at the cellular level, responses to chemotherapy have been demonstrated, thus defying the traditional belief held at an oncological level [6].…”

“…DT accounts for about 3% of soft tissue tumors, about 15% of which are extra-peritoneal DT [6]. The majority of patients present with painless swelling.…”

Nationwide Trends in the Current Management of Desmoid (Aggressive) Fibromatosis

“…Desmoid fibromatosis is rare, with an incidence of just 0.2e0.4/100 000 [1], although it carries a significant morbidity and a recognised mortality. The condition has a well-documented preponderance for females (2:1) and, although it can affect patients of a wide age range, is most commonly found in those aged 25e35 years [2].…”

Treatment of Aggressive Pelvic Fibromatosis With Interferon

“…It does not invade organs, but it will displace them and can lead to functional disorder emanating from fascial or deep musculoaponeurotic structures. [1][2][3][4][5][6][7][8] Surgical resection is the standard approach; however, this can have a high morbidity. In the current report, we describe a case of pelvic fibromatosis that was filling the vagina.…”