The epidemiology, demographic, clinical, treatment, and healthcare resource utilization (HRU) characteristics of desmoid tumor (DT) patients treated at two sarcoma centers in Denmark is described.
Using Danish health registers, we studied DT patients treated at two sarcoma centers between 2009 and 2018. For each patient, ten persons from the general population were randomly matched on birth year, sex, and region of residence.
Of the 179 DT patients identified, 76% were female and the median patient age was 38 years at diagnosis (interquartile range: 31–50). An average annual incidence of DTs over the study period was 3.2 per 1000,000 individuals with the observed annual incidence of DTs ranging from 2.2 (2011) to 4.3 (2017) per 1000,000 individuals. No notable linear time trend in incidence was observed. Anatomical DT sites included extra-abdominal (49%), abdominal wall (40%), and intra-abdominal or retroperitoneal areas (8%). In total, 56% of patients were initially treated surgically. However, while 75% of patients diagnosed with DT between 2009 and 2014 were initially treated surgically, this was true for only 32% of patients diagnosed with DT between 2015 and 2018. A total of 56% of DT patients used chemotherapeutic agents, tyrosine kinase inhibitors, NSAIDs, opioids, antidepressants, or steroids at some point during the three years before their DT diagnoses. In contrast, 70% of surgically treated and 63% of non-surgically treated patients used one of these drugs in the subsequent three years, including NSAIDs (45% surgical vs. 33% non-surgical), opioids (39% surgical vs. 27% non-surgical), and steroids (22% surgical vs. 18% non-surgical). The average number of inpatient and outpatient visits, days of hospitalization, and additional surgical procedures were higher among DT patients than the comparison cohort.
DTs are rare but have a large impact on patients’ health, HRU, and medication utilization.
- • The incidence of DTs exhibited no notable linear time trend in Denmark between 2009 and 2018.
- • A typical DT patient was a female in her fourth decade of life.
- • At sarcoma centers, initial treatment has shifted from surgery to active surveillance.
- • HRU was substantially higher in DT patients than in the general population.
Desmoid tumors (DTs), or aggressive fibromatosis, are rare, clonal soft tissue neoplasms characterized by locally aggressive fibroblastic proliferation, significant morbidity, and no metastatic potential . The estimated annual incidence of DTs in the general population is 2–5 cases per million . Individuals between the ages of 15 and 60 are most commonly affected .
The etiology of DTs is multifactorial. Most DTs (85–90%) arise sporadically. DTs also develop in about 10–25% of patients with familiar adenomatous polyposis (FAP) . One case series suggests up to thirty percent of DT patients have a history of trauma at the anatomical site where the DT arises , including surgery such as Caesarean section. High estrogenic states, pregnancy, and female sex have been reported as risk factors, leading to a two-to-three times higher prevalence of DTs in females . Common anatomic DT sites include the abdominal wall, limbs, girdles, and the mesenteric area. FAP patients frequently experience intra-abdominal DTs .
According to the 2018 European Society of Medical Oncology (ESMO) and the 2020 US National Comprehensive Cancer Network (NCCN) guidelines for soft tissue and visceral sarcomas , treatments may include surgery, isolated limb perfusion, cryoablation, radiotherapy, or systemic medical therapies, including chemotherapeutic agents, tyrosine kinase inhibitors (TKIs), non-steroidal anti-inflammatory drugs (NSAIDs), and hormonal therapies like tamoxifen for progressing cases.
Historically, total resection has been the mainstay DT treatment, but post-surgical recurrence rates are between 22% and 76% . These procedures may be highly morbid, causing potential disfigurement and functional impairment. By contrast, an initial active surveillance approach of monitoring the DT radiographically prior to committing to resection did not negatively impact outcomes but lowered overall morbidity in stable, asymptomatic cases . The first regional (European) recommendations and global consensus guidelines to advise initial active surveillance among certain patients were released in 2014 and 2020, respectively. Interest in avoiding debilitating surgical interventions is increasing.
Due to the rarity and complex clinical heterogeneity of DTs, high-quality evidence regarding their etiology, treatment, and prognosis is limited. Herein, we describe the epidemiology, demographic, clinical, survival, and healthcare resource utilization (HRU) characteristics of DT patients compared to a matched comparison cohort from the general population.