Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor) Journal Article


Authors: de Camargo, V. P.; Keohan, M. L.; D'adamo, D. R.; Antonescu, C. R.; Brennan, M. F.; Singer, S.; Ahn, L. S.; Maki, R. G.
Article Title: Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor)
Abstract: BACKGROUND: In the current study, the authors examined the outcomes of patients with desmoid tumors who received systemic therapy at a single institution to provide a basis for the examination of newer agents. METHODS: Records of patients with desmoid tumors who were treated with chemotherapy at the study institution were reviewed. The activity of nonsteroidal anti-inflammatory drugs was not addressed. Patients without measurable disease and those receiving therapy could not be documented, and those receiving prophylactic therapy were excluded. RESULTS: A total of 68 patients received 157 lines of therapy. At the time of last follow-up, 9 patients had died, 7 of progressive disease. The cohort was 62% female, with a median age of 32.5 years. Approximately 32% of the patients had Gardner syndrome. The median follow-up was 63 months, and patients received a median of 2 lines of therapy. An intra-abdominal primary tumor location was the most common (44%). The greatest Response Evaluation Criteria in Solid Tumors (RECIST) response rate was observed with anthracyclines and hormonal therapy and the lowest response was noted with single-agent dacarbazine/temozolomide or tyrosine kinase inhibitors, principally imatinib. On multivariate analysis, macroscopic nodular morphology and the presence of Gardner syndrome were the only tumor factors found to be associated with a greater time to disease progression. CONCLUSIONS: Compared with other agents, antiestrogens and anthracycline-containing regimens appear to be associated with a higher radiological response rate against desmoid tumors. Systemic therapy can be successful in patients with desmoid tumors, and is a viable option in lieu of morbid or disabling surgery. © 2010 American Cancer Society.
Keywords: adolescent; adult; cancer survival; controlled study; treatment outcome; treatment response; aged; middle aged; major clinical study; doxorubicin; cancer growth; systemic therapy; antineoplastic agents; chemotherapy; methotrexate; temozolomide; imatinib; dacarbazine; recurrence; medical record review; myelodysplastic syndrome; disease progression; ibuprofen; nonsteroid antiinflammatory agent; sulindac; tamoxifen; muscle disease; hormonal therapy; anthracycline; cardiomyopathy; desmoid tumor; retrospective analysis; fibromatosis, aggressive; deep fibromatosis; novel erythropoiesis stimulating protein; gardner syndrome; heart papillary muscle dysfunction
Journal Title: Cancer
Volume: 116
Issue: 9
ISSN: 0008-543X
Publisher: Wiley Blackwell  
Date Published: 2010-05-01
Start Page: 2258
End Page: 2265
Language: English
DOI: 10.1002/cncr.25089
PUBMED: 20187095
PROVIDER: scopus
PMCID: PMC2925106
DOI/URL:
Notes: --- - "Cited By (since 1996): 2" - "Export Date: 20 April 2011" - "CODEN: CANCA" - "Source: Scopus"
Altmetric Score
MSK Authors
  1. Murray F Brennan
    782 Brennan
  2. David R D'Adamo
    37 D'Adamo
  3. Cristina R Antonescu
    612 Antonescu
  4. Robert Maki
    211 Maki
  5. Mary Louise Keohan
    66 Keohan
  6. Linda Su Hyun Ahn
    8 Ahn
  7. Samuel Singer
    233 Singer