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- W2279662372 abstract "10519 Background: Aggressive fibromatosis (AF) or desmoid tumors are monoclonal proliferations which are locally invasive but do not metastasise. Sporadic tumors are usually associated with mutations in the beta-catenin gene CTNNB1whereas those occurring in the context of familial adenomatous polyposis usually have inactivating mutations in APC. Histologically they are characterised by nuclear expression of beta-catenin. When surgery and radiotherapy are not applicable or fail to control the disease, systemic treatment with anti-oestrogens, non steroidal anti-inflammatory drugs (NSAIDs) and chemotherapy can be used. A variety of regimens are reported to have activity including methotrexate/vinblastine and doxorubicin/dacarbazine. Recent reports indicate that single agent pegylated liposomal doxorubicin (Caelyx, C) is also effective. Methods: Ten patients with AF received C between June 2006 and December 2008. C was administered intravenously at 50 mg/m 2 over 1 hour every 4 weeks. Results: The female/male ratio was 9:1 and the median age at presentation was 39.5 years (range 18–53). All patients had progressive fibromatosis. Fifty percent had previously been treated with surgery/radiotherapy or both. All but one had previous systemic therapy which comprised tamoxifen/toremifene (6), NSAIDs (1), chemotherapy (1) and imatinib (1). C was well tolerated with mucositis (6/10), palmar-plantar erythema (4/10) and fatigue (2/10) being the main toxicities. A dose reduction to 40 mg/m 2 was required in 50% of cases hence the optimal dose lies between 40 and 50 mg/m 2 . One patient is currently receiving treatment and is too early to assess. For the nine patients who have completed treatment the median number of C cycles was 6 (range 4–6). Objective response according to RECIST was achieved in 4/10 patients and in 5 patients the best response was stable disease. Clinical benefit (pain relief, improved mobility) was observed in all patients. The duration of response ranged from 4 to 28 months. Conclusions: This is the largest series of patients with AF receiving C presented to date. C as single agent therapy has acceptable toxicity and highly promising activity in unresectable AF and may provide long term clinical benefit in some patients. No significant financial relationships to disclose." @default.
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- W2279662372 date "2009-05-20" @default.
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- W2279662372 title "Treatment of aggressive fibromatosis with pegylated liposomal doxorubicin: The Royal Marsden Hospital experience" @default.
- W2279662372 doi "https://doi.org/10.1200/jco.2009.27.15_suppl.10519" @default.
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