Occurrence of desmoid tumours in familial adenomatous polyposis and results of treatment

Abstract

Desmoids are of major concern in patients with familial adenomatous polyposis (FAP) besides the cancer risk. We estimated the risk for desmoid tumours and the results of their treatment in the Finnish Polyposis Registry. The analysis included 202 FAP patients, of whom 169 had undergone colectomy. Desmoids were observed in 29 cases: 15 in the mesentery, 10 in the abdominal wall and 4 in other sites. There were 12 male and 17 female patients with a mean age of 28.2 years (range 7 months to 59 years). The cumulative life-time risk was 21%; 1.5, 3.0, 8.9, 16 and 18% at ages of 10, 20, 30, 40 and 50 years, respectively. The risk of postcolectomy desmoids was 17% after ileorectal anastomosis and 12% after proctocolectomy. There were no deaths due to desmoids. One abdominal scar desmoid disappeared spontaneously and all the other abdominal wall desmoids could be excised without complications, but recurrence occurred in five (45%) cases. Excision was possible in only nine mesenteric desmoids (56%); in these cases recurrence was less common (two cases, 22%) but two others had life-threatening complications (bleeding, short bowel). We conclude that the desmoid problem concerns more than 20% of all FAP patients in long-term. Despite high recurrence rates and surgical hazards surgery remains a useful option for most desmoid tumours in FAP patients considering that other treatments are often ineffective or hazardous as well.

Gardner Syndrome: case report and brief review of the literature

“…5 O tumor desmóide, achado extraintestinal de maior relevância clínica na paciente em questão, é uma lesão fibrosa benigna, porém localmente agressiva, com predomínio na parede abdominal ou mesentério. Origina-se de estruturas musculoaponeuróticas, 9 não possui cápsula e potencialmente infiltra tecido muscular e fáscias adjacentes, com baixo potencial de metastatização. 6,10 O crescimento tumoral sofre influência hormonal pelo estrogênio, sendo o tumor desmóide mais frequente em pacientes do sexo feminino, 10 como no presente caso.…”

Desmoid tumor of the supraclavicular region: a case report

“…There is a female predominance while the highest incidence arises among patients between the ages of 15 to 60 years old [5]. Desmoids arise sporadically, nevertheless in an incidence ranging from 3% to 35% these tumors can occur in patients with familial adenomatous polyposis [6,7]. …”

Low-dose dacarbazine-doxorubicin therapy against intra-abdominal desmoid tumors

“…Surgery is usually indicated for extraabdominal desmoids such as those in the abdominal wall, but it is not recommended for mesenteric desmoid tumors, which account for the majority of intra-abdominal desmoids, since they are difficult to curatively resect. Surgery carries a high risk of serious complications such as bleeding, injury of the involved organs and short bowel syndrome, and it is associated with a high incidence of recurrence (6,7).…”