A case of Hand-Schüller-Christian disease with a poor response to treatment

We describe a 37-year-old woman with Hand- Schuller-Christian disease. She was given the diagnosis by a neurosurgeon because of diabetes insipidus and eosinophilic granuloma of the temporal bone. She subsequently had pain in the mandible. She was given a diagnosis of eosinophilic granuloma in the mandible by an oral surgeon. She underwent curettage of the tumor and received chemotherapy with vinblastine sulfate, etoposide, and prednisolone as well as radiotherapy. Despite treatment, the tumor repeatedly recurred. She was operated on nine times by neurosurgeons and twice by oral surgeons during the past 8 years. She is free of disease 20 months after the latest operation.