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This retrospective multicenter study aimed to analyze the clinical features and prognosis of 24 patients diagnosed with LGMS between 2002 and 2019 in the Japanese sarcoma network. Twenty-two cases were surgically treated and two cases were treated with radical radiotherapy (RT). The pathological margin was R0 in 14 cases, R1 in 7 cases, and R2 in 1 case. The best overall response in the two patients who underwent radical RT was one complete response and one partial response. Local relapse occurred in 20.8% of patients. Local relapse-free survival (LRFS) was 91.3% at 2 years and 75.4% at 5 years. In univariate analysis, tumors of 5 cm or more were significantly more likely to cause local relapse (p < 0.01). In terms of the treatment of relapsed tumors, surgery was performed in two cases and radical RT was performed in three cases. None of the patients experienced a second local relapse. Disease-specific survival was 100% at 5 years. A wide excision aimed at the microscopically R0 margin is considered the standard treatment for LGMS. However, RT may be a viable option in unresectable cases or in cases where surgery is expected to cause significant functional impairment.

BackgroundThe active surveillance (AS) has been suggested for the initial treatment of extra-abdominal desmoid fibromatosis (EADF), the percentage of patients who shifted to invasive secondary treatments was significant. Tranilast, an anti-keloid medication, is frequently used in Japan, but there is no detailed report on its efficacy.MethodsWe retrospectively analyzed the medical records of EADF patients treated with tranilast between January 2009 and March 2021. Since EADF has been reported to shrink spontaneously, the effects of all drugs should be compared to AS. Therefore, we compared its clinical course with that under AS (determined by a systematic review) to assess the effect of tranilast. A systematic literature review of AS outcomes was conducted on July 22, 2021, according to PRISMA guidelines. The primary endpoint was the rate of conversion to secondary treatment. Secondary endpoints were progression-free survival, objective response rate (ORR), disease control rate (DCR), and adverse events. The rates of conversion to secondary treatment, ORRs, and DCRs were compared between the two groups using the Fisher's exact test.ResultsEighteen patients who received tranilast as the initial treatment for EADF were included. Two patients (11.1%) underwent surgical resection due to tumor growth and continuous pain. Compared to the secondary treatment conversion rate from the pure AS approach (40.1%), tranilast was shown to significantly reduce the rate (p = 0.01). The ORR and DCR did not different between the two groups.ConclusionsAs initial treatment for EADF, tranilast is preferred over AS.