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

Early experience: high-intensity focused ultrasound treatment for intra-abdominal aggressive fibromatosis of failure in surgery

“…7,8 However, large intra-abdominal aggressive fibromatoses usually infiltrate adjacent structures and result in high local failure rates. 2,3,9 To our knowledge, there are no published examples of HIFU being used to treat intraabdominal aggressive fibromatosis after surgical failure.…”

Desmoid tumor mimics local recurrence of extremity sarcoma on MRI

“…Regarding first‐line treatment, Briand et al 32 showed the effectiveness of a “wait and see” approach to desmoid tumors. Several other studies corroborate the value of nonoperative desmoid tumor management 33‐36 . Because locally recurrent sarcoma would be treated with surgery and possible adjuvant treatment, it is critical that surgeons are aware of postresection desmoid formation and how to distinguish it from malignancy.…”

Surgical management of a retroperitoneal pelvic desmoid tumour involving the sacrifice of external iliac vein and internal iliac vessels

“…There is increased risk with extra-abdominal tumours, positive surgical margins and patients with FAP syndrome (Litchman, 2008 & Al-Jazrawe et al, 2015). Presently, there are no reliable genomic or clinical markers that predict tumour behaviour in regards to recurrence or regression and there is a growing field of research investigating this (Eastley et al, 2015). Regarding adjunctive therapy to reduce recurrence radiotherapy is possibly useful and targeted therapies focussing on the PDGFRβ pathway have been explored but response rates are low (Al-Jazrawe et al, 2015).…”

“…Desmoid tumours have an incidence of 0.2–0.4/100,000 and present at any age, most commonly between 25 and 35 years (Eastley et al, 2015). They represent less than 2% of all soft tissue sarcomas (Litchman, 2008).…”