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Observation
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Because of the variable natural history of desmoid tumors, as outlined above, observation is sometimes a viable option. This is particularly the case for lesions that are asymptomatic, do not pose a danger to vital organs, or are incompletely resected.[11,13-19]
A global consensus meeting that involved sarcoma experts with experience in both adult and pediatric desmoid tumor was organized to define the appropriate management of these tumors. The Desmoid Tumor Working Group suggested that an initial active surveillance approach does not influence the efficacy of subsequent treatments. They suggested that active therapy should only be considered in cases of persistent progression or symptoms. Active surveillance includes continuous monitoring with a first magnetic resonance imaging within 1 to 2 months of diagnosis, followed by scans in 3- to 6-month intervals. When the disease is located in critical structures that may pose significant morbidity, such as the mesentery and head and neck region, earlier treatment decisions should be made.[20]
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Evidence (observation vs. initial surgery):
A nonrandomized prospective cohort study included 771 patients with desmoid-type fibromatosis who were referred for second opinion and molecular analysis from 22 referral centers in France. The study compared initial surgery with initial observation.[21][Level of evidence C2]
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A nonrandomized prospective cohort study included 771 patients with desmoid-type fibromatosis who were referred for second opinion and molecular analysis from 22 referral centers in France. The study compared initial surgery with initial observation.[21][Level of evidence C2]
There was no difference in event-free survival (EFS) rates between the two groups (53% vs. 58%; P = .415).
A nonrandomized prospective cohort study included 771 patients with desmoid-type fibromatosis who were referred for second opinion and molecular analysis from 22 referral centers in France. The study compared initial surgery with initial observation.[21][Level of evidence C2]
Among patients with favorable tumor locations (defined as abdominal wall, intra-abdominal, breast, digestive viscera, and lower limb), the 2-year EFS rate was similar in patients who underwent surgery (70%) or were observed (63%; P = .41).
A nonrandomized prospective cohort study included 771 patients with desmoid-type fibromatosis who were referred for second opinion and molecular analysis from 22 referral centers in France. The study compared initial surgery with initial observation.[21][Level of evidence C2]
Among patients with tumors in unfavorable locations (defined as chest wall, head and neck, and upper limb), the 2-year EFS rate was significantly better for those treated nonsurgically (52%) compared with those who underwent initial surgery (25%; P = .001).
There were 173 patients with desmoid-type fibromatosis who were treated on European paediatric Soft Tissue Sarcoma Study Group (EpSSG) studies since 2005. Thirteen patients (8%) had biopsies only (no further treatment), 65 patients (42%) received chemotherapy only, 31 patients (20%) underwent surgery only, 36 patients (23%) had both chemotherapy and surgery, and 9 patients (6%) received radiation therapy in addition to other therapies.[22][Level of evidence C2]
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There were 173 patients with desmoid-type fibromatosis who were treated on European paediatric Soft Tissue Sarcoma Study Group (EpSSG) studies since 2005. Thirteen patients (8%) had biopsies only (no further treatment), 65 patients (42%) received chemotherapy only, 31 patients (20%) underwent surgery only, 36 patients (23%) had both chemotherapy and surgery, and 9 patients (6%) received radiation therapy in addition to other therapies.[22][Level of evidence C2]
All patients were alive at the time of analysis.
There were 173 patients with desmoid-type fibromatosis who were treated on European paediatric Soft Tissue Sarcoma Study Group (EpSSG) studies since 2005. Thirteen patients (8%) had biopsies only (no further treatment), 65 patients (42%) received chemotherapy only, 31 patients (20%) underwent surgery only, 36 patients (23%) had both chemotherapy and surgery, and 9 patients (6%) received radiation therapy in addition to other therapies.[22][Level of evidence C2]
The authors concluded that the conservative nonsurgical approach did not compromise outcome in pediatric patients.
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Preserved source evidence · Not medical advice