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. (May 2010) Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer, 116 (9). pp. 2258-65. ISSN 0008-543X (Print)0008-543X (Linking)
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.
| Item Type: | Paper |
|---|---|
| Uncontrolled Keywords: | Adolescent Adult Aged Antineoplastic Agents/*therapeutic use Disease Progression Female Fibromatosis, Aggressive/*drug therapy/mortality/radiography Humans Male Middle Aged Recurrence Treatment Outcome |
| Subjects: | diseases & disorders > cancer diseases & disorders > cancer > drugs and therapies |
| CSHL Authors: | |
| Communities: | CSHL labs > Maki lab |
| Depositing User: | Matt Covey |
| Date: | 1 May 2010 |
| Date Deposited: | 25 Oct 2016 18:49 |
| Last Modified: | 25 Oct 2016 18:49 |
| PMCID: | PMC2925106 |
| Related URLs: | |
| URI: | https://repository.cshl.edu/id/eprint/33707 |
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