Treatment and Follow-Up Strategies in Desmoid Tumours: A Practice Guideline

Abstract

Objectives: We set out to determine the optimal treatment options—surgery, radiation therapy (RT), systemic therapy, or any combinations thereof—for patients with desmoid tumours once the decision to undergo active treatment has been made (that is, monitoring and observation have been determined to be inadequate); provide clinical-expert consensus opinions on follow-up strategies in patients with desmoid tumours after primary interventional management. Methods: This guideline was developed by Cancer Care Ontario’s Program in Evidence-Based Care and the Sarcoma Disease Site Group. The MEDLINE, EMBASE, and Cochrane Library databases, main guideline Web sites, and abstracts of relevant annual meetings (1990 to September 2012) were searched. Internal and external reviews were conducted, with final approval by the Program in Evidence-Based Care and the Sarcoma Disease Site Group. Recommendations: Treatments: Surgery with or without RT can be a reasonable treatment option for patients with desmoid tumours whose surgical morbidity is deemed to be low; The decision about whether RT should be offered in conjunction with surgery should be made by clinicians and patients after weighing the potential benefit of improved local control against the potential harms and toxicity associated with RT; Depending on individual patient preferences, systemic therapy alone or RT alone might also be reasonable treatment options, regardless of whether the desmoid tumours are deemed to be resectable. Follow-Up Strategies: Undergo evaluation for rehabilitation (occupational therapy or physical therapy, or both); Continue with rehabilitation until maximal function is achieved; Undergo history and physical examinations with appropriate imaging every 3–6 months for 2–3 years, and then annually.

Management strategies in Lynch syndrome and familial adenomatous polyposis: a national healthcare survey in Japan

“…The last section consisted of questions regarding FAP (Table 3), including: number of FAP patients undergoing follow-up, number of FAP patients diagnosed in 2011-2015, number of patients receiving preventive surgery in 2011-2015 overall or by laparoscopic procedure, (31) whether the operation was carried out in their own hospital or elsewhere, surgical procedures, such as proctocolectomy or colectomy, proctocolectomy with hand-sewn or stapled ileal-anal anastomosis, (32,33) construction of pouch or not, type of pouch in case of pouch construction, (23,24,34) timing of operation, use of NSAIDs such as sulindac, (35) and number of patients with desmoid tumor in 2011-2015, type of desmoid tumor, and management. (20)(21)(22)36) Data collection. We asked all departments that belonged to JSCCR to reply to the questionnaire from April 7 to May 13, 2016 using e-mail and letter.…”

“…(4,5,11,19) The rare desmoid tumor is one of the common diseases that accompanies FAP. (5,(19)(20)(21)(22) Abdominal desmoid tumor is especially intractable because of its high recurrence rate after surgery.Data about hereditary CRC in Japan are limited. (10,11) Our department has developed the surgical procedure of…”

Invasion of the Anterior Abdominal Wall: A Case Report of a Desmoid Tumour

“…This has resulted in a lack of consensus in the literature regarding the most appropriate treatment for DF. The Cancer care Ontario's program and sarcoma disease group recommend a watch and wait approach for suitable patients who have a stable, non-aggressive lesion which is slow growing [7]. However, in a clinical setting, given the rareness of these desmoid tumours it would be difficult to differentiate this from a malignant tumour.…”

Assessment of Physician's Systemic Treatment Preferences for Patients with Advanced Desmoid-Type Fibromatosis: Experience-Based Medicine in the Absence of High-Level Evidence

“…The true efficacy of these treatments cannot be assessed, due to the occurrence of spontaneous regressions in DF and due to publication bias. Proposed treatments for patients with advanced DF include a broad spectrum of agents, including anti-inflammatory agents, anti-hormonal drugs, cytokines, chemotherapy, and targeted agents [1][2][3][4][5][8][9][10]. The fact that these drugs do not have a common mechanism of action raises doubts about their true efficacy, and their relative therapeutic value remains to be further elucidated.…”

“…Hardly any of the commonly used therapeutic options for localized or more advanced disease have been assessed rigorously in large prospective clinical trials. Randomized evidence in support of a specific therapy is more or less non-existent [1][2][3][4][5][8][9][10]. Watchful waiting, surgery, and radiotherapy are typical treatment options for localized DF.…”