NAİL PAKSOY, FERHAT FERHATOGLU, IZZET DOGAN, NAZİYE AK, METİN PEHLİVAN, MELTEM EKENEL, MERT BASARAN
Eurasian Journal of Medical Investigation - 2022;6(3):326-331
Objectives: Desmoid tumors (DTs) are rare benign neoplasms characterized by histologically monoclonal fibroblastic proliferation. Although current treatment guidelines recommend active surveillance as the initial approach, systemic therapy should be considered in rapidly progressive or symptomatic patients. In this study, we aimed to evaluate the efficacy of the kinase inhibitor, sorafenib, as a treatment for patients with progressive or symptomatic DTs. Methods: The clinical, pathological, and demographic data of a sample of patients treated for DTs with sorafenib were retrospectively evaluated. Results: Seventeen patients were included in the study. The ratio of female to male patients was 2.4, and the median age was 32 (range: 14-65). Four (23.5%) patients had Gardner syndrome. The rates of extra-abdominal and ıntraintra abdominal tumors were 64.7% and 35.3%, respectively. The median follow-up duration before sorafenib treatment be gan was 6 years. Before sorafenib, 15 patients had undergone surgical resection. All patients had received a median of two lines of systemic therapy, and four (23.5%) patients had received chemotherapy. The median sorafenib treatment duration was 23.4 months. The 1 and 2 year progression-free survival rates were 94.1% and 80.7%, respectively. Grade 34 toxicities were observed in six (35.2%) of the patients. Conclusion: Sorafenib was deemed an effective treatment for previously treated advanced DTs.
