Even though they do not metastasize, desmoid tumors often interfere with everyday activities and can be life-threatening1,2
Desmoid tumor characteristics:2-5
- Rare
- Locally aggressive
- Unpredictable clinical course
- Vital organs can be impacted
Desmoid tumors are associated with a potentially high and multifaceted burden of illness:6,*
- Pain
- Disfigurement
- Decreased physical function
In focus groups and interviews, patients reported that pain was the most debilitating symptom of desmoid tumors.1,†
In medical literature and clinical practice, desmoid tumors may also be referred to as aggressive fibromatosis, desmoid-type fibromatosis, or deep fibromatosis
Examples of desmoid tumors and potential symptoms
Back
Large desmoid tumor (15 cm x 10 cm) proximal to the spine7
Hand
Desmoid tumor causing severe restriction in the flexion of the hand8
Knee
MRI scan showing desmoid tumor behind the right knee associated with electric paresthesias and reduced flexion9
Neck
CT scan showing desmoid tumor in the right upper neck involving the brachial plexus associated with pain, numbness, and weakness in the right arm10
Data from a Memorial Sloan Kettering/Desmoid Tumor Research Foundation patient-reported outcome (PRO) validation study included patients with desmoid tumors (n=31, age range 20-68, 77% female). Patients participated in 60-minute qualitative phone interviews to provide their perspectives on disease symptoms and impact on their quality of life. The majority of the patients in this study were symptomatic (84%). Tumor site and type varied across patients. The concepts discussed during interviews were used to develop a draft patient-reported outcome scale, which was further refined in cognitive interviews of additional patients with desmoid tumors (n=15).6
Twenty-seven patients with desmoid tumors were interviewed from the Royal Marsden Hospital in the United Kingdom. Two focus groups and 13 interviews explored health-related quality of life issues and experiences of healthcare related to their desmoid tumors.1
CT, computed tomography; MRI, magnetic resonance imaging.
Desmoid tumor management is evolving
Clear margins are often challenging to achieve with surgery and may require extensive resection that can lead to additional pain and functional impairment.11-14
Surgery is no longer recommended as first-line treatment for most clinical situations15,16
- Up to 77% recurrence rates after surgical resection of desmoid tumors13,17,‡
- In a retrospective analysis, extremity, chest wall, and intra-abdominal desmoid tumors were associated with higher risk of recurrence than tumors located at other sites18
- Independent studies have reported desmoid tumor recurrence rates of up to 30% even with clear margins18-21
- Surgical trauma and growth factors released during wound healing may worsen desmoid tumors and promote recurrence12,13,22
Systemic therapies are recommended as a first-line treatment option for progressive, morbid, or symptomatic desmoid tumors, according to the NCCN Guidelines® and Desmoid Tumor Working Group (DTWG) Guideline.15,16,§
Based on retrospective, observational data. Factors associated with local recurrence postsurgery include tumor location, age of the patient, tumor size, margin status, and prior recurrence.18,23
NCCN Guidelines also recommend ablation therapy procedures and definitive radiation therapy as first-line treatment options for progressive, morbid, or symptomatic desmoid tumors for certain patients.15
Identifying the earliest signs of desmoid tumor progression is key for patient management
NCCN Guidelines and DTWG Guideline recommendations for initiating treatment15,16,24
- Symptoms worsening
- Tumor growth documented on imaging (eg, MRI or CT)
- Impairing functioning or daily activities
CT, computed tomography; MRI, magnetic resonance imaging; NCCN, National Comprehensive Cancer Network® (NCCN®).
Give your patients with desmoid tumors a chance to respond to a new systemic treatment