Long-Term Outcomes for Desmoid Tumors Treated With Radiation Therapy

Guadagnolo, B. Ashleigh; Zagars, Gunar K.; Ballo, Matthew T.

doi:10.1016/j.ijrobp.2007.10.013

Cited by 131 publications

(116 citation statements)

References 22 publications

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“…These and our findings are in contrast with the effectiveness of radiotherapy reported in adult series. 30 The possible reasons for these different results are unclear and may be only objects of speculation (ie, more infiltrative pattern of AF in children than in adults, different biology, or different Original Article aggressiveness of pediatric vs adult radiotherapists). In fact, the use of radiotherapy needs to be limited in children because of the greater risk of iatrogenic sequelae (functional morbidity and second malignancies) than in adults, and it probably deserves a role only in certain situations, ie, after chemotherapy has failed in patients with unresectable disease, in the event of progression despite multiple surgeries, or as an alternative to mutilating surgery.…”

Section: Discussionmentioning

confidence: 99%

Aggressive fibromatosis in children and adolescents

Meazza¹,

Bisogno

Gronchi³

et al. 2009

Cancer

108

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BACKGROUND:Aggressive fibromatosis (AF) is a rare tumor of intermediate malignancy that has a strong potential for local invasiveness and recurrence. To date, there are no general recommendations for the clinical management of pediatric AF.METHODS:The authors retrospectively analyzed 94 patients aged ≤21 years, including 23 patients who underwent complete surgery (Group I), 42 patients who underwent incomplete surgery with microscopic residual tumor (Group II), and 29 patients who underwent either biopsy or macroscopically incomplete surgery (Group III).RESULTS:The 5‐year event‐free survival (EFS) and overall survival rates were 44% and 99%, respectively. Local recurrences developed in 22% of patients in Group I, in 76% of patients in Group II, and in 76% of patients in Group III. Two of 7 patients with abdominal disease died of tumor progression, whereas none of the patients with extra‐abdominal AF died of their disease. Systemic treatment was given to 15 patients as first‐line treatment and to 34 patients at time the time they developed recurrent disease: The response rate was 47% in the former patients and 50% in the latter patients. Objective responses were observed in 11 of 19 patients who received combined methotrexate plus vinblastine/vinorelbine, in 7 of 15 patients who received alkylating‐agent chemotherapy, and in 4 of 11 patients who received other therapies (tamoxifen, sulindac, interferon alfa).CONCLUSIONS:The current analysis suggested that the clinical course of AF in children may resemble that of AF in adults. Local recurrences did not affect the chance of responding to systemic therapy or the survival rate. The completeness of initial resection was the main factor that influenced EFS, whereas disease control after marginal resection was much the same as that achieved after intralesional surgery/biopsy. Good responses to systemic treatments, and particularly to low‐dose chemotherapy, were observed as reported previously in adults. Cancer 2010. © 2010 American Cancer Society.

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Section: Discussionmentioning

confidence: 99%

Aggressive fibromatosis in children and adolescents

Meazza¹,

Bisogno

Gronchi³

et al. 2009

Cancer

108

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“…Four studies indicated that younger age (≤30 years in three studies 12,18,23 , ≤18 in one study 11 ) was predictive of a higher local failure rate.…”

Section: Key Evidencementioning

confidence: 97%

“…Of five retrospective comparative studies that conducted a multivariate analysis [10][11][12]18,23 , one did not find a significant difference in patient characteristics at baseline 26 . Three single-arm phase ii studies of systemic therapy 16,24,29 serve as the primary evidence base for the recommendations.…”

Section: Key Evidencementioning

confidence: 99%

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Treatment and Follow-Up Strategies in Desmoid Tumours: A Practice Guideline

et al. 2014

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Objectives: We set out to determine the optimal treatment options—surgery, radiation therapy (RT), systemic therapy, or any combinations thereof—for patients with desmoid tumours once the decision to undergo active treatment has been made (that is, monitoring and observation have been determined to be inadequate); provide clinical-expert consensus opinions on follow-up strategies in patients with desmoid tumours after primary interventional management. Methods: This guideline was developed by Cancer Care Ontario’s Program in Evidence-Based Care and the Sarcoma Disease Site Group. The MEDLINE, EMBASE, and Cochrane Library databases, main guideline Web sites, and abstracts of relevant annual meetings (1990 to September 2012) were searched. Internal and external reviews were conducted, with final approval by the Program in Evidence-Based Care and the Sarcoma Disease Site Group. Recommendations: Treatments: Surgery with or without RT can be a reasonable treatment option for patients with desmoid tumours whose surgical morbidity is deemed to be low; The decision about whether RT should be offered in conjunction with surgery should be made by clinicians and patients after weighing the potential benefit of improved local control against the potential harms and toxicity associated with RT; Depending on individual patient preferences, systemic therapy alone or RT alone might also be reasonable treatment options, regardless of whether the desmoid tumours are deemed to be resectable. Follow-Up Strategies: Undergo evaluation for rehabilitation (occupational therapy or physical therapy, or both); Continue with rehabilitation until maximal function is achieved; Undergo history and physical examinations with appropriate imaging every 3–6 months for 2–3 years, and then annually.

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“…Four studies included patients with primary desmoid tumours 8,12,17,32 . Only one retrospective study compared the main clinical characteristics of the patients at baseline, showing no significant differences between the intervention groups 25 ; however, five studies conducted a multivariate analysis to control for potential confounders at baseline [9][10][11]17,22 . Only two studies reported a blinded assessment of outcome 23,28 .…”

Section: 22mentioning

confidence: 99%