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. Cancer 2010;116:2258-65.

Results of surgical treatment in patients with abdominal desmoid fibromas

“…Чаще применяют гормональную и цитостатическую тера-пию. Анализ литературы показал, что имеется до 157 раз-личных комбинаций лекарственного лечения [32,33].…”

“…Чаще применяют в ка-честве первой линии при нерадикальных операциях либо при прогрессировании заболевания [31,[33][34][35][36].…”

Triple Therapy with Doxorubicin, Imatinib and Tamoxifen in Recurrence Desmoids Tumor Associated with Pregnancy: Case Report and Literature Review

“…Based therapy regarding response rates. In the series of de Camargo, anthracyclines in combination with hormonal therapy demonstrated the greatest RECIST response rates (6). Endocrine and/or NSAID therapy is often considered first-line medical treatment for unresectable, advanced disease without clinical symptoms (7).…”

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

“…However, there has been no controlled randomized trial in this setting. The influence of chemotherapy in the treatment of the desmoid tumors is unclear and limited (2,7,18,28). However, some retrospective series and the French sarcoma group study demonstrated good outcomes with chemotherapy (29).…”