***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
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    Input: PURPOSE This study compared a large series of desmoid patients treated at a single institution to a previously published series from the same institution to determine if patient population characteristics , treatment approaches , and clinical outcomes had undergone change over the two study periods . MATERIAL S AND METHODS Data from a prospect i ve soft tissue tumor data base was used to analyze clinical courses of 189 desmoid patients treated at The University of Texas M.D. And erson Cancer Center ( UTMDACC ) from 1995 to 2005 as compared with 189 UTMDACC desmoid patients treated between 1965 and 1994 . RESULTS A nearly three-fold increase in annualized UTMDACC desmoid referral volume with significantly higher percentages and numbers of primary desmoid tumor referrals to UTMDACC was observed in the most recent study period . Significantly increased systemic therapy use and decreased reliance on surgery alone was observed more recently . While the recent series patients had higher rates of macroscopic residual disease and equivalent rates of positive microscopic margins after definitive surgery , the estimated 5-year local recurrence rate of 20 % was improved compared with the 30 % rate observed in the earlier series . CONCLUSION Increased awareness of the complex multidisciplinary management needed for desmoid tumor control may underlie significantly increased numbers of referrals to UTMDACC , especially primary untreated desmoids . Increased neoadjuvant treatments may be associated with improved desmoid patient outcomes . These trends should be supported , particularly if personalized molecular-based therapies are to be rapidly and effectively deployed for the benefit of those afflicted by this rare and potentially debilitating disease Flaws in the design , conduct , analysis , and reporting of r and omised trials can cause the effect of an intervention to be underestimated or overestimated . The Cochrane Collaboration ’s tool for assessing risk of bias aims to make the process clearer and more Purpose Surgery is still the st and ard treatment for desmoid-type fibromatosis ( DF ) . Recently , the Institut Gustave Roussy ( IGR ) , Villejuif , France , reported a series of patients treated with a front-line conservative approach ( no surgery and no radiotherapy ) . The disease remained stable in more than half of patients . This study was design ed to evaluate this approach on the natural history of the disease in a larger series of patients . Methods A total of 142 patients presenting to the IGR or Istituto Nazionale Tumori ( INT ) , Milan , Italy , were initially treated using a front-line deliberately conservative policy . Their progression-free survival ( PFS ) was observed and a multivariate analysis was performed for major clinical variables . Results Seventy-four patients presented with primary tumor , 68 with recurrence . Eighty-three patients received a “ wait & see ” policy ( W&S ) , whereas 59 were initially offered medical therapy ( MT ) , mainly hormonal therapy and chemotherapy . A family history of sporadic colorectal cancer was present in 8 % of patients . The 5-year PFS was 49.9 % for the W&S group and 58.6 % for the medically treated patients ( P = 0.3196 ) . Similar results emerged for primary and recurrent DF . Multivariate analysis identified no clinical variables as independent predictors of PFS . In the event of progression , all patients were subsequently managed safely . Conclusions A conservative policy could be a safe approach to primary and recurrent DF , which could avoid unnecessary morbidity from surgery and /or radiation therapy . Half of patients had medium-term stable disease after W&S or MT . A multidisciplinary , stepwise approach should be prospect ively tested in DF PURPOSE We report the treatment of 10 children for progressive desmoid tumor not amenable to st and ard surgical or radiation therapy with the use of vinblastine ( VBL ) and methotrexate ( MTX ) . PATIENTS AND METHODS Ten patients aged 6.4 to 18 years with primary ( two patients ) or recurrent ( eight patients ) desmoid tumor were treated with VBL and MTX for 2 to 35 months . Patients with recurrent tumors had been previously treated with surgical resection with ( two patients ) or without ( five patients ) radiation therapy or with radiation therapy alone ( one patient ) . No patient had previously received cytotoxic chemotherapy . The tumor response was assessed at routine intervals by physical examination and magnetic resonance imaging ( MRI ) . RESULTS Five patients had clinical evidence of response to therapy with complete resolution ( three patients ) or partial resolution ( two patients ) of physical examination and radiographic abnormalities . Three patients had stable disease during 10 to 35 months of treatment . Two of these patients had progressive disease 9 and 37 months after treatment stopped ; one patient had no progression 16 months after therapy . Two additional patients with stable disease had chemotherapy discontinued after 2 and 3 months . Common side effects included mild alopecia and myelosuppression and moderate nausea and vomiting . In patients with responding tumors , MRI showed decreased tumor size and , in two patients , changes consistent with fibrosis and decreased cellularity of the tumor . CONCLUSION Combination chemotherapy with VBL and MTX appears to control desmoid tumor without significant acute or long-term morbidity in most children . This may allow for further growth and development in these patients , which may decrease the morbidity of subsequent definitive therapy PURPOSE To determine the efficacy and safety of using vinblastine ( Vbl ) and methotrexate ( Mtx ) in children with desmoid-type fibromatosis that is recurrent or not amenable to treatment with radiation or surgery . PATIENTS AND METHODS A phase II study was conducted within the Pediatric Oncology Group . Patients were treated using Vbl ( 5 mg/m2/dose ) and Mtx ( 30 mg/m2/dose ) , both administered by intravenous injection weekly for 26 weeks and every other week for an additional 26 weeks . Response was assessed by bidimensional measurements of tumor on axial imaging ( magnetic resonance imaging or computed tomography ) . RESULTS Over 35 months , 28 patients were enrolled ; 27 were eligible , and 26 were assessable for response . A measurable response was documented in eight patients ( 31 % ) , and 10 patients had stable disease documented as the best response to treatment . Eighteen patients had disease progression at a median time of 9.1 months . Eight patients remain free of disease progression at a median of 43.4 months from study entry . Nine patients reported no to moderate toxicity . Neutropenia was the most common toxicity ( n = 22 ) and the most common grade 4 toxicity ( n = 5 ) . Anemia , nausea , vomiting , and elevations in hepatic transaminases were also common and were reversible with interruption of chemotherapy . CONCLUSION Vbl and Mtx are well tolerated in children with desmoid-type fibromatosis . Furthermore , this combination can promote tumor regression or block tumor growth in most children Abstract Background . Aggressive fibromatosis ( AF ) is a locally invasive proliferative disease . The mainstay of treatment is surgery . Chemotherapy may be considered in inoperable AF following failure of hormonal therapy and /or NSAIDs . Material and methods . We conducted a retrospective search of the prospect ively maintained Royal Marsden Hospital Sarcoma Unit data base to identify patients with AF treated with chemotherapy between 1987 and 2009 . Results . Thirty-nine patients , thirty one females and eight males , received one or more lines of chemotherapy . The most frequently employed chemotherapy regimens were methotrexate/vinblastine [ MTX/VBL ] ( 18 ) and pegylated liposomal doxorubicin [ PLD ] ( 14 ) . MTX/VBL was administered weekly or every two weeks at MTX 50 mg and VBL 10 mg . Treatment duration ranged from three weeks to one year with a median of 4.5 months . Partial response ( PR ) was observed in 11 % of cases , disease stabilisation ( SD ) in 60 % and progressive disease ( PD ) in 22 % . Time to progression ranged from one month to sixteen years . The main toxicities reported were mucositis ( 4 ) , peripheral neuropathy ( 3 ) , vomiting ( 3 ) , and neutropenia ( 3 ) . PLD was administered at 40–50 mg/m2 every four weeks , for up to six cycles . PR was achieved in 33 % and in the remainder the disease was stable with no progression during treatment . Three ( 25 % ) patients have so far progressed after treatment . Symptomatic benefit , especially pain relief , was reported in 86 % ( 12/14 ) of cases . Main toxicities included palmar plantar erythema ( 5 ) and mucositis ( 4 ) . Discussion . MTX/VBL remains a useful combination but PLD is emerging as a well tolerated and effective systemic therapy in advanced AF This Phase II study was undertaken to assess the activity of methotrexate plus vinblastine in the treatment of patients with inoperable aggressive fibromatosis ( AF ) and to observe the evolution of the disease after such low‐dose chemotherapy Background This study was conducted to determine the efficacy and safety of low-dose chemotherapy with methotrexate ( MTX ) and vinblastine ( VBL ) for patients with desmoid tumors refractory to meloxicam treatment , focusing in particular on the relationship between the efficacy of this chemotherapy and catenin β-1 ( CTNNB1 ) mutation status . Patients and methods Since March 2003 , patients pathologically diagnosed with extraperitoneal desmoid tumors have been prospect ively treated with meloxicam  , a COX-2 inhibitor  , at our institution . Patients with inoperable tumors who were resistant to meloxicam treatment underwent MTX and VBL therapy every other week . The responses of all patients were evaluated , and factors that were correlated with efficacy were analyzed , including CTNNB1 mutation status . Results Sixty-eight patients were prospect ively treated with meloxicam . MTX + VBL therapy was administered in 15 patients . Six patients showed a partial response . Only one patient presented disease progression . A few patients showed grade 3–4 treatment-related toxicity with the administration of MTX and VBL every other week . Intriguingly , CTNNB1 status did not affect the efficacy of this treatment . Conclusion MTX and VBL treatment every other week is well tolerated and achieved a favorable response in patients resistant to meloxicam treatment , regardless of CTNNB1 mutation status We have ( 1 ) estimated the incidence of desmoid tumor ( DT ) in the Finnish population , and ( 2 ) defined statistically four major age components of the DT with different biological properties . The incidence of the DT , based on admissions to four separate hospitals and on the number of pathological biopsy specimens analyzed at the Central Pathological Laboratory of Helsinki University , is 2.4 - 4.3 new cases per 10(6 ) inhabitants per annum . Statistical analysis demonstrated four major age components where the site of the tumor and /or sex of the patient were non-r and omly distributed : " juvenile " DT , a predominantly extra-abdominal desmoid tumor of the female sex ; " fertile " DT , a nearly exclusively abdominal DT of fertile females ; " menopausal " DT , a predominantly abdominal tumor where the sex ratio approaches one : one ; and " senescent " DT , where abdominal and extra-abdominal varieties are equally frequently encountered and where the sex ratio of the affected patients is one : one 
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The efficacy of this chemotherapy was convincing .
However , the overall evidence was weak , and this chemotherapy is not covered by insurance in Japan ; we only weakly recommend low-dose chemotherapy with methotrexate and vinblastine in patients with extra-abdominal desmoid-type fibromatosis