The treatment of desmoid tumors: a stepwise clinical approach

Bonvalot, Sylvie; Desai, A.; Coppola, Sara; Péchoux, C. Le; Terrier, Philippe; Dômont, Julien; Cesne, Axel Le

doi:10.1093/annonc/mds298

“…It reduces the incidence of local recurrence for these patients and it may control unresectable disease (13,14). Concurrently, some authors have reported a wait and see policy for stable and asymptomatic lesions, while others have recommended chemotherapy or systemic therapy for unresectable lesions (2,7,15,16).…”

mentioning

confidence: 99%

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

Ergen

¹

,

Tiken

²

,

Öksüz

³

et al. 2016

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Background: Desmoid tumors are uncommon and benign mesenchymal neoplasms. The optimal treatment of patients with desmoid tumors is still controversial. Surgery is the primary treatment for locally invasive or recurrent desmoid tumors. Also, radiotherapy is a treatment option for patients at high risk for local failure such as those with positive margins or recurrent and unresectable tumors. Aims: To report our institutional experience and longterm results of patients with desmoid tumors who received radiotherapy. Study Design: Retrospective cross-sectional study. Methods: Between 1980 and 2009, 20 patients who received radiotherapy (RT) in our institution were analyzed. The majority of patients (80%) were referred with a recurrent tumor after previous surgery. Thirteen patients underwent marginal resection, 4 had wide local excision and 3 patients had only biopsy. Resection margin was positive in 15 (75%) patients. All patients received radiation therapy. The median prescribed dose was 60 Gy. Five patients received less than 54 Gy. Results: The median follow-up time was 77.5 months (28-283 months). Nine patients developed local recurrence after RT. Seven local failures (78%) were in field. Time to local recurrence ranged from 3-165 months (median 33 months). The 2-5 year local control (LC) rates were 80% and 69%, respectively. On univariate analysis, the 5 year local control rate was significantly better in the patients treated with ≥54 Gy than in patients who received <54 Gy (p=0.023). The most common acute side effect was grade 1-2 skin toxicity. As a late side effect of radiotherapy, soft tissue fibrosis was detected in 10 patients and lymphangitis was seen in 1 patient. One patient developed radiation-induced sarcoma. Conclusion: According to our results, radiotherapy is especially effective in recurrent disease and provides a high local control rate in the patients received more than 54 Gy.

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“…However, there has been no controlled randomized trial in this setting. The influence of chemotherapy in the treatment of the desmoid tumors is unclear and limited (2,7,18,28). However, some retrospective series and the French sarcoma group study demonstrated good outcomes with chemotherapy (29).…”

Section: Discussionmentioning

confidence: 99%

“…It is a rare non-malignant soft tissue neoplasm. (2,3). The etiology of the disease is unknown, but trauma, postsurgical scar, endocrine and genetic causes are being considered as possible risk factors for the development of desmoid tumors (3,4).…”

mentioning

confidence: 99%

“…The etiology of the disease is unknown, but trauma, postsurgical scar, endocrine and genetic causes are being considered as possible risk factors for the development of desmoid tumors (3,4). Desmoid tumors have a wide spectrum of heterogeneity in clinical attitude, but they usually have an infiltrative growth and locally aggressive behavioral pattern, and do not distantly metastasize (2,5,6). These tumors have been classified as intermediate grade sarcomas since they are locally invasive and local recurrence rates are very high after wide local resection (7,8).…”

mentioning

confidence: 99%

See 1 more Smart Citation

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

Ergen¹,

Tiken²,

Öksüz³

et al. 2016

View full text Add to dashboard Cite

Background: Desmoid tumors are uncommon and benign mesenchymal neoplasms. The optimal treatment of patients with desmoid tumors is still controversial. Surgery is the primary treatment for locally invasive or recurrent desmoid tumors. Also, radiotherapy is a treatment option for patients at high risk for local failure such as those with positive margins or recurrent and unresectable tumors. Aims: To report our institutional experience and longterm results of patients with desmoid tumors who received radiotherapy. Study Design: Retrospective cross-sectional study. Methods: Between 1980 and 2009, 20 patients who received radiotherapy (RT) in our institution were analyzed. The majority of patients (80%) were referred with a recurrent tumor after previous surgery. Thirteen patients underwent marginal resection, 4 had wide local excision and 3 patients had only biopsy. Resection margin was positive in 15 (75%) patients. All patients received radiation therapy. The median prescribed dose was 60 Gy. Five patients received less than 54 Gy. Results: The median follow-up time was 77.5 months (28-283 months). Nine patients developed local recurrence after RT. Seven local failures (78%) were in field. Time to local recurrence ranged from 3-165 months (median 33 months). The 2-5 year local control (LC) rates were 80% and 69%, respectively. On univariate analysis, the 5 year local control rate was significantly better in the patients treated with ≥54 Gy than in patients who received <54 Gy (p=0.023). The most common acute side effect was grade 1-2 skin toxicity. As a late side effect of radiotherapy, soft tissue fibrosis was detected in 10 patients and lymphangitis was seen in 1 patient. One patient developed radiation-induced sarcoma. Conclusion: According to our results, radiotherapy is especially effective in recurrent disease and provides a high local control rate in the patients received more than 54 Gy.

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“…Therefore, desmoid tumor assigns a group of soft tissue neoplasms in which the deep fibromatosis, or aggressive fibromatosis, is highlighted. It is originated by clonal proliferation of fibroblasts with high replication capacity, what confers tendency to local invasion, as well as to recurrence, although histologic changes and biomarkers of aggressiveness are not seen [3].…”

Section: Introductionmentioning

confidence: 99%

Desmoid Tumor of Rectus Abdominis: Recommendations for the Treatment and Reconstruction of the Parietal Defect

Gomes

¹

2014

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The desmoid tumor is a connective tissue's neoplasm, derived from fibroblasts, characterized by the absence of histologic changes or biomarkers of aggressiveness. It has a tendency to local invasion as well as to recurrence, and has been described in association with familial adenomatous polyposis (Gardner's syndrome). This disease prevails in pregnant women, during menacme, or during the postpartum. The biologic behavior of the desmoid tumor is unpredictable, which enables heterogeneity in its clinical course. Sometimes, it is possible to observe tumors with a high tendency to relapse even after an adequate surgery with safety margin resection. In other cases, initial growth is followed by a stable clinical course despite any therapeutic modality. When large and located at the rectus abdominis muscle, the surgical treatment represents a challenge, once a monoblock resection leads to severe defects on the abdominal wall. The difficulties found during the reconstruction require experienced teams and, in the majority of cases, the use of fasciocutaneous flaps or news special meshes. We report a case of a large desmoid tumor located at the upper and medium third of the left rectus abdominis, in a 41 years old, Afro-descendent, female patient, submitted to curative resection of the entire muscle segment and simultaneous reconstruction of the abdominal wall by the insertion of a polypropylene light mesh (15 × 12 cm). The patient had an uneventful recovery and was discharged within fourth postoperative days. Clinical control after one year of follow-up showed neither recurrence nor functional or aesthetic complications.

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The treatment of desmoid tumors: a stepwise clinical approach

Cited by 142 publications

References 45 publications

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

Desmoid Tumor of Rectus Abdominis: Recommendations for the Treatment and Reconstruction of the Parietal Defect

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