Desmoid tumors: To treat or not to treat, That is the question

Abstract

Desmoid tumors (DTs) are a rare disease of intermediate malignancy characterized histologically by a locally aggressive, monoclonal, fibroblastic proliferation and clinically by a variable and often unpredictable course. For decades, surgical resection has been the standard initial treatment approach; however, more recently, a paradigm shift toward a more conservative treatment strategy has been introduced. More than 5 years ago, The Desmoid Tumor Working Group started a consensus initiative in Europe with the aim of harmonizing the strategy among clinicians and setting up treatment recommendations for patients with DTs. This review summarizes the latest joint, global, evidence-based guideline approach to DT management. Moreover, a number of gray areas in the treatment recommendations are discussed, and possible future perspectives on the treatment armamentarium for patients with DTs are presented.

Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors

“…Forth, we were not able to compare the event-free survival between first-line observation and medical treatment groups due to the small sample size. Kasper et al stated that a stepwise therapy escalation from an initial, less toxic treatment including observation to more toxic agents seems reasonable for progressive DTs [29]. These limitations should be considered in the analysis of our study results.…”

“…According to a recent systematic literature review of active surveillance for patients with DTs, the median follow-up time of the patients was reported in 12 studies and ranged from 8 to 73 months [28]. In the case of subsequent progression or a significant increase in symptom burden, a decision toward treatment may be considered and assessed with at least 3 further assessments and possibly not before 1 year after the initial diagnosis [29]. This study showed poor extremity functional outcomes in the first-line observation/medical treatment group.…”

Surgery in Soft Tissue Sarcoma: the Thin Line between a Surgical or more Conservative Approach

“…Although, for many years, surgery was the standard primary treatment option for desmoid tumors, a more conservative management approach has recently been adopted. The current primary approach is active surveillance, with surgery considered only after failure of medical therapies [1,2]. The benefits of a conservative approach were first identified in two retrospective studies of desmoid tumors.…”

The Evaluation of Health-Related Quality of Life Issues Experienced by Patients with Desmoid-Type Fibromatosis (The QUALIFIED Study)—A Protocol for an International Cohort Study

“…Although this tumour does not metastasise, its potential locally aggressive tumour growth can cause significant morbidity. It is therefore classified as an intermediate tumour by the World Health Organisation [1,2]. The biological behaviour of DTF is unpredictable and displays phases of progressive growth or growth stabilisation and up to 28% of tumours ultimately undergo spontaneous regression [3][4][5].…”