Active surveillance in desmoid-type fibromatosis: A systematic literature review

Abstract

Background: This study evaluates the results of the active surveillance (AS) approach in adult patients with desmoid-type fibromatosis (DTF) because AS is advocated as a front-line approach for DTF in the European consensus guidelines. Methods: A systematic literature search was conducted (December 19th, 2019, updated on April 14th, 2020. Studies describing the outcomes of the AS approach were included. The PRISMA guidelines were used. Results: Twenty-five articles were included for data retrieval. Forty-two percent of reported patients (1480 of 3527 patients) received AS, the majority were women and the majority had a primary tumour. The median age at diagnosis ranged from 28 to 59 years. Common tumour sites were the extremities/girdles (n Z 273), the abdominal wall (n Z 253) and the trunk (n Z 153). The median reported percentage of progressive disease, stable disease and partial response was 20% (interquartile range [IQR]: 13e35%), 59% (IQR: 37e69%) and 19% (IQR 3e23%), respectively. In 640 patients, the outcome was not specified. The median reported percentage of shifting to an active form of treatment was 29%, most commonly to systemic treatment (n Z 195) and surgery (n Z 107). The reported median follow-up time ranged between 8 and 73 months. The reported median time to progression and/or initiation of the subgroup shifting from AS to 'active' therapy ranged from 6.3 months to 19.7 months. Conclusion:The majority of patients undergoing AS have either stable disease or a partial response, and about one-third of patients shift to an active form of treatment. Selecting

Management of Patients with Newly Diagnosed Desmoid Tumors in a First-Line Setting

“…Additionally, only approximately one third of patients initially managed with active surveillance experienced worsening pain or progressive tumor growth, requiring either medical treatment or local therapy. These results overall suggest that initial active surveillance should be preferred, whenever possible, to a primary surgical approach, which is in line with current guidelines and available evidence [ 33 , 34 , 35 ]. Indeed, while surgery followed by adjuvant radiotherapy correlated with increased PFS and TTNT compared to active surveillance, the morbidity associated with surgery and the risk of secondary malignancies related to radiotherapy must be both taken into consideration.…”

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

“…Indications for active treatment include pain, with or without radiological evidence of progression, functional symptoms, or patient request [27]. 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].…”

Clinical features and long‐term outcomes of aggressive fibromatosis of the abdominal wall after surgical resection: A retrospective study and literature review

“…Selecting patients who will benefit from active surveillance upfront should be the priority of future studies. 26 If the tumor progresses during follow-up period, the treatment strategy needs to be altered. A Canadian practice guideline for the treatment of AF recommended that surgery with or without radiation therapy can be a reasonable treatment option for patients with AF whose surgical morbidity is low.…”