Chemotherapy, for unresectable or recurrent tumors

Methotrexate and vinblastine: This combination produced objective responses in about one-third of patients with unresectable or recurrent desmoid-type fibromatosis.[23]

Doxorubicin and dacarbazine: A series of mainly adult patients with FAP and unresectable desmoid-type fibromatosis that were unresponsive to hormone therapy showed that doxorubicin plus dacarbazine followed by meloxicam (an NSAID) can be safely administered and can induce responses.[24]

Pegylated liposomal doxorubicin: In a study of 11 patients, 4 patients achieved an objective response and 7 patients had stable disease.[25] In a series of five patients, a median progression-free interval of 29 months was reported.[26]

Hydroxyurea: A retrospective analysis reported the results of 16 children with previously treated desmoid tumors who were treated with hydroxyurea. Before hydroxyurea, seven patients had tumor progression, two patients had increased pain, and seven patients had both. Tumor shrinkage occurred in 37.5% of patients (with 18.7% partial remissions), and symptom improvement occurred in 68.7% of patients.[27]

Vinorelbine: A retrospective review of 24 patients with desmoid-type fibromatosis (median age, 13.9 years; range, 1–23 years) received oral vinorelbine at eight centers of the Société Française des Cancers de l'Enfant between 2005 and 2020. For the 23 evaluable patients, 13% had partial responses, 78% had disease stabilization, and 9% had disease progression. The progression-free survival (PFS) rate was 89.3% at 24 months.[28]