From the Guidelines
For Desmond tumor follow-up, a wait-and-see policy can be advised in tumors which are not life-threatening or asymptomatic [III, B] 1. This approach is based on the recent clinical practice guidelines for soft tissue sarcoma and GIST, which suggest that a wait-and-see policy is a viable option for Desmoid-type fibromatosis (DF) tumors that are not causing symptoms or are not life-threatening 1. Some key points to consider in the follow-up of Desmond tumor include:
- Regular clinical examinations by an oncologist or a multidisciplinary team with expertise in sarcoma management
- Imaging studies such as MRI scans to monitor tumor size and progression
- Assessment for symptoms such as pain, neurological changes, or constitutional symptoms like unexplained weight loss or fatigue
- Consideration of systemic therapy, such as sorafenib or pazopanib, if the tumor is symptomatic or progressive 1
- Discussion of treatment options, including surgery, radiation, or chemotherapy, in a multidisciplinary team setting It is essential to note that the follow-up plan may need to be adjusted based on the individual patient's risk factors, tumor type, and stage. The goal of follow-up is to detect any changes in the tumor early, allowing for prompt intervention and improving treatment outcomes. In cases where systemic therapy is feasible, options like sorafenib and pazopanib have shown to improve progression-free survival (PFS) over placebo and methotrexate-vinblastine, respectively, in randomized trials 1.
From the Research
Desmond Tumor Follow-Up
- Desmond tumor, also known as desmoid tumor or aggressive fibromatosis, is a rare monoclonal neoplasm that arises from deep musculoaponeurotic structures 2.
- The optimal follow-up strategy for desmoid tumors involves history and physical examinations with appropriate imaging every 3-6 months for 2-3 years, and then annually 3.
- Treatment options for desmoid tumors include surgery, radiation therapy, systemic therapy, or a combination of these approaches 3.
- Systemic therapy options currently include hormonal agents, nonsteroidal anti-inflammatory drugs, tyrosine kinase inhibitors, and anthracycline-based regimens 4.
- Imatinib mesylate has been shown to be effective in the treatment of desmoid tumors, with a non-progression rate of 91% at 3 months and 67% at 12 months 5.
- Other tyrosine kinase inhibitors, such as sorafenib, are also being used to treat desmoid tumors, although they may have limited efficacy and significant side effects 4.
- New investigational therapies, including nirogacestat, AL102, and Tegavivint, are being studied for the treatment of desmoid tumors, and may offer new possibilities for patients with this disease 4.
- Rehabilitation, including occupational therapy and physical therapy, is an important part of the follow-up strategy for desmoid tumors, and can help patients achieve maximal function 3.