A 10-year review of surgery for desmoid disease associated with familial adenomatous polyposis

Abstract

Surgery for intra-abdominal desmoids in selected patients is less hazardous than previously reported. Surgery for abdominal wall and extra-abdominal tumours is safe. However, disease recurrence remains a major problem.

Diagnosis and multidisciplinary treatment of sporadical desmoid tumors

“…Five year local recurence rates of 8 to 44% were reported by other series, and was 36% in the present study (2,34,110,114,115,137,182,184). Most recurrences are usually observed within 3 years, and nearly all by 6 years and m ost of the studies found that the presence of positive surgical margin proved to be relevant in patients with recurrent disease (19,34,110,114,115,137,(182)(183)(184). Well corresponding with these results, the median time to recurrence in our series was 26 months (range 6-110 months) and the investigator established that obtaining a wide disease-free margin is crucial for reducing the recurrence rate.…”

“…Radical excision is often a therapeutic challenge or even impossible for intra-abdominal desmoids, confirmed by previous and recent studies which reported a rather low success rate of radical removal of intra-abdominal or retroperitoneal desmoids (8,32,91,107,(113)(114)(115)(116).…”

“…Due to the lack of randomized, controlled trials, we have tried to extrapolate from the published series those cases with tumors localized to the abdominal wall to compare with our findings (2,19,34,110,114,115,137,(182)(183)(184). Table 22.…”

“…Currently, a stepwise approach is recommended. Less toxic modalities such as antihormonal therapy or NSAIDs are preserved for patients with disease without any impending threat to life or function (7,112,115,142). The next stage is cytotoxic chemotherapy for fast growing desmoids not responsive to first-line agents (8).…”

Japanese Society for Cancer of the Colon and Rectum (JSCCR) Guidelines 2016 for the Clinical Practice of Hereditary Colorectal Cancer (Translated Version)

“…They often develop in the abdominal wall, mesentery or retroperitoneum after (procto)colectomy (in particular, within 2 to 3 years) 26,34) , and when developing intraabdominally (including in the retroperitoneum), they can cause bowel obstruction, perforation, abscess formation, ureteral obstruction, etc., often making treatment difficult. The mortality rate of FAP patients developing desmoid tumors is reported to be 0-14% 26,83,118,120,121) .…”