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" |
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