Abstract: Objective To characterize the clinical features of desmoid tumor, assess the efficacy of conservative chemotherapy
for inoperable desmoid tumor and analyze the prognostic factors. Methods From August 2009 to December 2013, 52 patients
with inoperable desmoid tumor were treated in our department and received chemotherapy with vinorelbine combined with
low-dose methotrexate. The clinical data of the patients were analyzed retrospectively. Results The patients studied included
22 male and 30 female patients with the age of disease onset ranging from 2 to 46 years (mean 18.7 years). The lesions occurred
most frequently in the lower limbs (36.5%, 19/52) and the tumor size ranged from 2.7 to 37 cm (mean 9.5 cm). The patients
were followed up for a median of 29 months (7 to 64 months). The chemotherapy lasted for 4 to 30 months (median 12
months). After completion of the chemotherapy, 1 patient had a complete response (CR), 18 showed partial responses (PR), 27
cases had stable disease (SD), and 6 had progressive disease (PD), with an overall response rate (ORR) of 88.5% . The
progression-free survival (PFS) time of the patients ranged from 4 to 63 months (median 26.5 months) with a 2-year PFS rate of
76.7% and 5-year PFS rate of 41.9%. A longer chemotherapy duration (over 12 months) was associated with a more favorable
prognosis. No significant differences in PFS were found between the patients stratified by gender, age of disease onset, age
when receiving chemotherapy, tumor site, or tumor size. Conclusion For recurrent, inoperable and progressive desmoid
tumor, long enough cycles of vinorelbine combined with low-dose methotrexate can be an effective and safe option for tumor
control.