Advanced aggressive fibromatosis: Effective palliation with chemotherapy.

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.

• Authors: Anastasia Constantinidou, Robin L Jones, Michelle Scurr, Omar Al-Muderis, Ian Judson
• Journal: Acta oncologica (Stockholm, Sweden) (Acta Oncol) Vol. 50 Issue 3 Pg. 455-61 (Apr 2011) ISSN: 1651-226X [Electronic] England
• PMID: 20799916 (Publication Type: Evaluation Study, Journal Article)

Chemical References

• liposomal doxorubicin
• Polyethylene Glycols
• Vinblastine
• Doxorubicin
• Methotrexate

Topics

• Abdominal Neoplasms (diagnostic imaging, drug therapy, pathology)
• Adolescent
• Adult
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Child
• Child, Preschool
• Disease Progression
• Doxorubicin (administration & dosage, analogs & derivatives)
• Female
• Fibromatosis, Aggressive (diagnostic imaging, drug therapy, pathology)
• Humans
• Male
• Methotrexate (administration & dosage)
• Middle Aged
• Neoplasm Invasiveness
• Palliative Care (methods)
• Polyethylene Glycols (administration & dosage)
• Radiography
• Registries (statistics & numerical data)
• Retrospective Studies
• Treatment Outcome
• Vinblastine (administration & dosage)
• Young Adult