Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients

Bonvalot, Sylvie; Eldweny, Hany; Haddad, Vincent; Rimareix, Françoise; Missenard, G.; Oberlin, Odile; Vanel, D.; Terrier, Philippe; Blay, Jean‐Yves; Cesne, Axel Le; Péchoux, C. Le

doi:10.1016/j.ejso.2007.06.006

Cited by 255 publications

(202 citation statements)

References 37 publications

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“…Several prognostic factors such as age, gender, tumor location and tumor size have been reported to play a role in the local control of the disease in literature (10,16,23,24). In the study by Melis et al (21), extremity lesions were associated with an increased recurrence.…”

Section: Discussionmentioning

confidence: 99%

“…The 5 year local control rate in patients received ≥54 Gy was 79%, which is comparable with the literature. Currently, some investigators also suggested an observation for stable and asymptomatic lesions since it has a slowly growing pattern and/or may spontaneously regress (7,15,16,23). In a recently published study, the authors declared that approximately 50% of patients with desmoid tumors benefit from non-aggressive treatment approaches.…”

Section: Discussionmentioning

confidence: 99%

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

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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

et al. 2016

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“…Sporadic extra-abdominal fibromatosis has a high tendency for local recurrence, even after apparently adequate resection. In institutional retrospective studies, local failure rates at 5 years have ranged from 25 to 60% (Bonvalot et al, 2008). This wide range reflects the great variability of accrual, treatments, and follow-up in this rare disease, which has not yet been investigated in a randomised controlled study.…”

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confidence: 99%

“…Traditionally, patients undergo standard surgery, with the primary goal always being to achieve complete resection with negative margins, as indicated for sarcomas. However, Bonvalot et al (2008) recently questioned the need for systematically including surgery and other aggressive treatments in the first-line treatment of primary extra-abdominal desmoid tumours. In their study, tumour growth arrest occurred in two-thirds of patients managed non-surgically.…”

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confidence: 99%

High frequency of β-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management

et al. 2010

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BACKGROUND: Fibromatosis comprises distinct clinical entities, including sporadic extra-abdominal fibromatosis, which have a high tendency for recurrence, even after adequate resection. There are no known molecular biomarkers of local recurrence. We searched for b-catenin mutations in a European multicentre series of fibromatosis tumours to relate b-catenin mutational status to disease outcome. METHODS: Direct sequencing of exon 3 b-catenin gene was performed for 155 frozen fibromatosis tissues from all topographies. Correlation of outcome with mutation rate and type was performed on the extra-abdominal fibromatosis group (101 patients). RESULTS: Mutations of b-catenin were detected in 83% of all cases. Among 101 extra-abdominal fibromatosis, similar mutation rates (87%) were observed, namely T41A (39.5%), S45P (9%), S45F (36.5%), and deletion (2%). None of the clinico-pathological parameters were found to be significantly associated with b-catenin mutational status. With a median follow-up of 62 months, 51 patients relapsed. Five-year recurrence-free survival was significantly worse in b-catenin-mutated tumours regardless of a specific genotype, compared with wild-type tumours (49 vs 75%, respectively, P ¼ 0.02). CONCLUSION: A high frequency (87%) of b-catenin mutation hallmarks extra-abdominal fibromatosis from a large multicentric retrospective study. Moreover, wild-type b-catenin seems to be an interesting prognostic marker that might be useful in the therapeutic management of extra-abdominal fibromatosis.

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“…Studies have shown no difference in survival between patients treated with surgery or radiation therapy compared with no treatment in this population (17,18). Surgical resection is indicated in those individuals with aggressive lesions.…”

Section: Discussionmentioning

confidence: 97%

Contralateral recurrence of aggressive fibromatosis in a young woman: A case report and review of the literature

2015

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Abstract. Aggressive fibromatosis (AF) is a benign non-encapsulated tumor of mesenchymal origin, with a tendency for local spread along fascial planes. Local invasion can lead to extensive morbidity and even mortality due to destruction of the bones, organs and soft tissues. This rare lesion is observed 1,000 times more frequently in patients with familial adenomatous polyposis or Gardner's syndrome due to the inheritance of the adenomatous polyposis coli (APC) gene. While AF does not metastasize, local recurrence is common. Distant recurrence is extremely rare, but is observed in those with a germ line APC mutation. The present study details the case of a 20-year-old woman with a melanoma of the right shoulder, treated definitively with surgery. The patient then developed a painful mass at the surgical site; a surgical biopsy demonstrated that the mass was AF. The patient was treated with surgical resection, radiation therapy, and a course of tamoxifen. Five years later, the patient presented with left forearm pain and diminished range of motion due to an infiltrating mass. This was excised and a clinical diagnosis of recurrent AF was made, in this patient lacking familial predisposition to the disease.

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Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients

Cited by 255 publications

References 37 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

High frequency of β-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management

Contralateral recurrence of aggressive fibromatosis in a young woman: A case report and review of the literature

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