The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network

Radiation Oncology, University Hospital Zurich, Switzerland.
Radiation Oncology (Impact Factor: 2.55). 02/2007; 2(1):12. DOI: 10.1186/1748-717X-2-12
Source: PubMed


A multi-centre study to assess the value of combined surgical resection and radiotherapy for the treatment of desmoid tumours.
One hundred and ten patients from several European countries qualified for this study. Pathology slides of all patients were reviewed by an independent pathologist. Sixty-eight patients received post-operative radiotherapy and 42 surgery only. Median follow-up was 6 years (1 to 44). The progression-free survival time (PFS) and prognostic factors were analysed.
The combined treatment with radiotherapy showed a significantly longer progression-free survival than surgical resection alone (p smaller than 0.001). Extremities could be preserved in all patients treated with combined surgery and radiotherapy for tumours located in the limb, whereas amputation was necessary for 23% of patients treated with surgery alone. A comparison of PFS for tumour locations proved the abdominal wall to be a positive prognostic factor and a localization in the extremities to be a negative prognostic factor. Additional irradiation, a fraction size larger than or equal to 2 Gy and a total dose larger than 50 Gy to the tumour were found to be positive prognostic factors with a significantly lower risk for a recurrence in the univariate analysis. This analysis revealed radiotherapy at recurrence as a significantly worse prognostic factor compared with adjuvant radiotherapy. The addition of radiotherapy to the treatment concept was a positive prognostic factor in the multivariate analysis.
Postoperative radiotherapy significantly improved the PFS compared to surgery alone. Therefore it should always be considered after a non-radical tumour resection and should be given preferably in an adjuvant setting. It is effective in limb preservation and for preserving the function of joints in situations where surgery alone would result in deficits, which is especially important in young patients.

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Available from: Brigitta G Baumert
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    • "The weighted kappa for overall agreement between reviewers for the final eligibility decision was 0.91 [95% CI (0.85–0.96)]. From this total of 35 papers, seven were categorized as radiation therapy (all retrospective design),6,15,16,18–22 18 as surgical therapy (2 prospective design; 16 retrospective design),2,3,5,8–11,13,18,22–30 and 14 were considered combination therapy (3 prospective; 11 retrospective design).3,6,8,10,12,13,18,19,22,26,28,30–32 Furthermore, nine were considered systemic therapy, four non-cytotoxic (3 prospective; 1 retrospective design),33–36 and 5 were cytotoxic (3 prospective; 2 retrospective design).7,37–40 "
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    ABSTRACT: The local control of desmoid tumors constitutes a continuing treatment dilemma due to its high recurrence rates. The purpose of this systematic review was to critically examine the current treatment of these rare tumors and to specifically evaluate the local failure and response rates of surgery, radiation and systemic therapy. We comprehensively searched the literature for relevant studies across Cinahl, Embase, Medline and the Cochrane databases. Articles were categorized as surgery, radiation, surgery + radiation and systemic therapy (including cytotoxic and non cytotoxic). Methodological quality of included studies was assessed using the Newcastle-Ottawa Scale. Pooled odd ratios (OR) for comparative studies and weighted proportions with 95% confidence intervals (CI) are reported. Thirty-five articles were included in the final analysis. Weighted mean local failure rates were 22% [95% CI (16–28%)], 35% [95% CI (26–44%)] and 28% [95% CI (18–39%)] for radiation alone, surgery alone and surgery + radiation respectively. In the analysis of comparative studies, surgery and radiation in combination had lower local failure rates than radiation alone [OR 0.7 (0.4, 1.2)] and surgery alone [OR 0.7 (0.4, 1.0)]. Weighted mean stable disease rates were 91% [95% CI (85–96%)] and 52% [95% CI (38–65%)] for non cytotoxic and cytotoxic chemotherapy respectively. The current evidence suggests that surgery alone has a consistently high rate of local recurrence in managing extra-abdominal desmoid tumors. Radiation therapy in combination with surgery improves local control rates. However, the limited data on systemic therapy for this rare tumor suggests the benefit of using both cytotoxic and non cytotoxic chemotherapy to achieve stable disease.
    Full-text · Article · Feb 2013 · Rare tumors
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    • "Combined treatment with surgery plus radiotherapy showed a significantly longer progression-free survival than surgical resection alone [42,47]. A study of 110 desmoid patients by Baumert confirmed that local control was significantly increased if radiotherapy was added to surgery, independent of tumour status and surgical margins [42]. Significantly better results are derived when post-operative adjuvant radiotherapy is added, compared to just radiotherapy at recurrence. "
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    ABSTRACT: Primary intra-thoracic desmoids are exceedingly rare borderline tumors, with 34 reported cases in the English-language literature. The characteristic localized infiltrative growth and the high rate of recurrence can result in life-threatening conditions. Radical surgical resection is considered to be the primary treatment. Achieving negative surgical margins is a challenge. Cases with positive surgical margins are associated with a high rate of local recurrence; therefore, other multimodal approaches play a large role in their therapy. The authors reviewed the relevant literature and presented examples of long-term follow-up of 3 intra-thoracic desmoid tumour patients, multidisciplinarily treated between 2000 and 2008. All reports of intra-thoracic desmoid tumors that the authors could find on PubMed or in the reference sections of these PubMed located articles were included using the search terms: intra-thoracic, desmoid, aggressive fibromatoses. Because of the rarity of the disease and the heterogeneity of the cases, it is difficult to assess the importance of the information for everyday clinical practice. It does however provide a useful guide for reference.
    Preview · Article · Feb 2012 · Medical science monitor: international medical journal of experimental and clinical research
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    • "It is complicated by the absence of any cleavage plane, and by adherence to neighboring structures; exeresis must therefore be either large-scale, entailing functional risk and morbidity, even amputation, or incomplete, entailing an elevated rate of recurrence [5]: overall 10-year recurrence is estimated at 77% [3]. Radiotherapy is recommended as an alternative when surgery is not feasible [5] and, more frequently, as an adjuvant in case of incomplete resection, although not in case of recurrence, in which it seems to be less effective [8]. The interest of treating these benign tumors with chemotherapy or other hormonal or anti-inflammatory agents or interferon-alpha, on the other hand, is controversial [5]. "
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    ABSTRACT: Hyperthermic isolated limb perfusion (ILP) (2 mg, TNF-alpha and 100 mg, melphalan) was performed for an irresectable right thigh desmoid tumor with calf extension in a 49-year-old man. The patient had a history of four resections since the age of 19 years. Local ILP toxicity appeared with extensive edema and common peroneal neurologic impairment including paresis that remained severe 10 months later. One of the most troublesome side effects of perfusion is peripheral nerve damage, which has been reported at a rate of between 1 and 48% of perfused patients. ILP is an effective treatment in recurrence situations or where resection threatens loss of function; it, however, requires administration in specialized centers, progress in standardization and close monitoring to avoid locoregional toxicity, the mechanisms of which merit further investigation. Emergency compartmental pressure measurement may indicate fasciotomy, can be of great interest.
    Full-text · Article · Dec 2009 · Orthopaedics & Traumatology Surgery & Research
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