Ifosfamide and doxorubicin were efficacious in a patient with advanced metastatic disease, producing significant disease regression. The addition of intrathecal or intraventricular therapy with hydrocortisone, ARA-C, and methotrexate was effective in controlling CNS disease due to chordoma. There was no apparent benefit from the use of actinomycin-D, cyclophosphamide and vincristine nor the combination of cisplatin and 5-fluorouracil or high-dose methotrexate.

No abstract

In patients with underlying hematologic disease such as Fanconi anemia, PFAPA syndrome may be associated with severe clinical problems in contrast to otherwise normal children with the disorder.