Table 4.
Short version: statements pertaining to desmoid tumours
| Statements | Level of evidence and agreement |
|---|---|
| Diagnosis and screening | |
| DTs.1: The different classifications can help in the choice of treatment; however, they must be strongly related to the clinical presentation and evaluation by the physician. | LE: low Agreement: 100% (SA 45%; A: 55%) |
| DTs.2: Preoperative screening for DT appears more relevant in patients who already had abdominal surgery as it might find a DT that can have impact on the surgical options choice. | LE: low Agreement: 90% (SA 28%; A: 62%; N 7%; D 3%) |
| DTs.3: There is no evidence in the literature that a screening programme for DT detection after abdominal surgery should be proposed. Moreover, with the actual possible treatment and the unpredictable evolution of DT such a screening programme might not be needed. | LE: low Agreement: 93% (SA 29%; A: 64%; N 3.5%; D 3.5%) |
| DTs.4: Confirmatory biopsies may be considered if there is a diagnostic dilemma or required to initiate medical therapy. | LE: very low Agreement: 92% (SA 71%; A: 21%; N 3.5%; D 4.5%) |
| DTs.5: In a patient with FTs without known FAP, screening of FAP (at least with colonoscopy and APC mutation testing if possible) should be performed. This is especially important among patients younger than 60 years, or with intra-abdominal desmoids or in the abdominal wall. | LE: Low Agreement: 89% (SA 46%; A: 43%; N 4%; D 7%) |
| Treatment | |
| DTs.6: Rapidly enlarging and life-threatening FT requires first-line aggressive treatment. Others should be surveyed in a watch-and-wait protocol. | LE: Low Agreement: 92% (SA 46%; A: 46%; N 8%) |
| DTs.7: Surgery should not be considered the ideal treatment for DTs, except in the case of DT complications, rapidly growing or life-threatening. | LE: Low Agreement: 89% (SA 30%; A: 59%; N 7%; D 4%) |
| DTs.8: There is currently no evidence to support the use of CP for high-risk patients undergoing surgery or in post-surgical care | LE: – Agreement: 88% (SA 35%; A: 53%; N 9%; D 3%) |
| Management for DTs identified during abdominal surgery | |
| DTs.9A: Continue with the intervention (proceeding with the surgical procedure) if technically feasible. | LE: Low Agreement: 92% (SA 21%; A: 71%; N 8%) |
| DTs.9B: Resection of mesenteric desmoid(s) should be avoided if it will result in sacrificing any small bowel. | LE: Low Agreement: 97% (SA 48.5%; A: 48.5%; N 3%) |
| DTs.10: Desmoid disease can potentially render restorative procedures technically challenging or impossible. In cases where it is feasible, restorative procedures should be cautiously considered and selectively recommended for patients with concomitant intra-abdominal DTs following prophylactic (procto)colectomy, taking into account the significant risk of desmoid recurrence and adhesion formation. In such circumstances, proctocolectomy with terminal ileostomy may represent the safest option. It is important to have a thorough discussion with the patients about the potential risks of compromised function and the possibility of requiring additional surgeries, ensuring that the choice is individualized to their specific situation. | LE: Low Agreement: 92% (SA 40%; A: 52%; N 8%) |
| DTs.11: The risk of DTs has not been evaluated on a systemic scale. When feasible, single-stage proctocolectomy is preferred for FAP patients in order to avoid DTs. | LE: — Agreement: 81% (SA 35%; A: 46%; N 12%; D 7%) |
A, agree; CP, chemoprevention; D, disagree; DT, desmoid tumour; FAP, familial adenomatous polyposis; LE, level of evidence; N, neutral; SA, strongly agree; SD, strongly disagree.