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Advanced aggressive fibromatosis: Effective palliation with chemotherapy

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Constantinidou, A., Jones, R. L., Scurr, M., Al-Muderis, O., Judson, I. (2011) Advanced aggressive fibromatosis: Effective palliation with chemotherapy. ACTA ONCOLOGICA, 50 (3). pp. 455-461. ISSN 0284-186X

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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 prospectively maintained Royal Marsden Hospital Sarcoma Unit database 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/m(2) 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.

Item Type: Article
Authors (ICR Faculty only): Judson, Ian
All Authors: Constantinidou, A., Jones, R. L., Scurr, M., Al-Muderis, O., Judson, I.
Additional Information: ISI Document Delivery No.: 734GY Times Cited: 0 Cited Reference Count: 24 Constantinidou, Anastasia Jones, Robin L. Scurr, Michelle Al-Muderis, Omar Judson, Ian Informa healthcare London
Uncontrolled Keywords: low-dose chemotherapy desmoid tumors radiation-therapy surgery methotrexate vinblastine mutations sarcoma
Research teams: ICR divisions > Cancer Therapeutics > Clinical Pharmacology & Trials (including Drug Metabolism & Pharmacokinetics Group)
Clinical Units > Sarcoma Unit
Depositing User: Barry Jenkins
Date Deposited: 08 Apr 2011 10:07
Last Modified: 10 Aug 2011 13:59
URI: http://publications.icr.ac.uk/id/eprint/10534

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The Royal Marsden - NHS foundation trust