Noncytotoxic drug therapy in children with unresectable desmoid tumors

Antiestrogens and nonsteroidal antiinflammatory drugs have been shown to be effective in adult patients with unresectable or recurrent desmoid tumors. It appears that the growth of these tumors is influenced by estrogen, and that antiestrogen treatment may inhibit further proliferation of tumor cells. Nonsteroidal antiinflammatory drugs are thought to be effective through their interference with prostaglandin metabolism.

Two children with unresectable desmoid tumors (aggressive fibromatosis) were treated with tamoxifen (1 mg/kg orally, twice daily) and diclofenac (2 mg/kg rectally, twice daily).

At last follow-up, tumor regression and growth arrest were maintained for more than 51 months in 1 child with rapidly growing recurrent fibromatosis of the thoracic wall. Another child with an inoperable desmoid tumor of the submandibular region had stable disease since the initiation of treatment.

This is the first report describing this treatment approach in childhood fibromatosis. Combined therapy with endocrine therapy and nonsteroidal antiinflammatory drugs may be a nonaggressive alternative to chemotherapy and radiotherapy in the treatment of children with inoperable desmoid tumors.