Skull lesions in the mastoid, temporal, or orbital bones

Chemotherapy. The current treatment for CNS-risk bones is 12 months of vinblastine/prednisone therapy, as per the HISTSOC-LCH-III (NCT00276757) study:[27,29][Level of evidence B1]There is controversy about whether systemic therapy is required for the first presentation of unifocal bone LCH, even in the CNS-risk bones. One retrospective review reported a series of patients with orbital or mastoid lesions who underwent only surgical curettage. The treatment was completed by a single surgeon, specialized in orbital, ear, nose, or throat diseases.[102] None of these patients developed diabetes insipidus. However, when comparing the incidence rates of diabetes insipidus in patients who received little or no chemotherapy (20%–50% incidence) with the incidence rates reported by the German-Austrian-Dutch group DAL-HX 83 trial (10% incidence in patients treated for LCH), it appears that the weight of evidence from the DAL-HX 83 trial supports chemotherapy treatment to prevent diabetes insipidus in patients with LCH in CNS-risk bones.[76,77] It should be noted, however, that the DAL-HX studies administered more drugs and treated patients for 12 months.

Chemotherapy. The current treatment for CNS-risk bones is 12 months of vinblastine/prednisone therapy, as per the HISTSOC-LCH-III (NCT00276757) study:[27,29][Level of evidence B1]There is controversy about whether systemic therapy is required for the first presentation of unifocal bone LCH, even in the CNS-risk bones. One retrospective review reported a series of patients with orbital or mastoid lesions who underwent only surgical curettage. The treatment was completed by a single surgeon, specialized in orbital, ear, nose, or throat diseases.[102] None of these patients developed diabetes insipidus. However, when comparing the incidence rates of diabetes insipidus in patients who received little or no chemotherapy (20%–50% incidence) with the incidence rates reported by the German-Austrian-Dutch group DAL-HX 83 trial (10% incidence in patients treated for LCH), it appears that the weight of evidence from the DAL-HX 83 trial supports chemotherapy treatment to prevent diabetes insipidus in patients with LCH in CNS-risk bones.[76,77] It should be noted, however, that the DAL-HX studies administered more drugs and treated patients for 12 months.

Weekly vinblastine (6 mg/m2) for 7 weeks for good response.

Chemotherapy. The current treatment for CNS-risk bones is 12 months of vinblastine/prednisone therapy, as per the HISTSOC-LCH-III (NCT00276757) study:[27,29][Level of evidence B1]There is controversy about whether systemic therapy is required for the first presentation of unifocal bone LCH, even in the CNS-risk bones. One retrospective review reported a series of patients with orbital or mastoid lesions who underwent only surgical curettage. The treatment was completed by a single surgeon, specialized in orbital, ear, nose, or throat diseases.[102] None of these patients developed diabetes insipidus. However, when comparing the incidence rates of diabetes insipidus in patients who received little or no chemotherapy (20%–50% incidence) with the incidence rates reported by the German-Austrian-Dutch group DAL-HX 83 trial (10% incidence in patients treated for LCH), it appears that the weight of evidence from the DAL-HX 83 trial supports chemotherapy treatment to prevent diabetes insipidus in patients with LCH in CNS-risk bones.[76,77] It should be noted, however, that the DAL-HX studies administered more drugs and treated patients for 12 months.

Daily prednisone (40 mg/m2) for 4 weeks, then tapered over 2 weeks.

Chemotherapy. The current treatment for CNS-risk bones is 12 months of vinblastine/prednisone therapy, as per the HISTSOC-LCH-III (NCT00276757) study:[27,29][Level of evidence B1]There is controversy about whether systemic therapy is required for the first presentation of unifocal bone LCH, even in the CNS-risk bones. One retrospective review reported a series of patients with orbital or mastoid lesions who underwent only surgical curettage. The treatment was completed by a single surgeon, specialized in orbital, ear, nose, or throat diseases.[102] None of these patients developed diabetes insipidus. However, when comparing the incidence rates of diabetes insipidus in patients who received little or no chemotherapy (20%–50% incidence) with the incidence rates reported by the German-Austrian-Dutch group DAL-HX 83 trial (10% incidence in patients treated for LCH), it appears that the weight of evidence from the DAL-HX 83 trial supports chemotherapy treatment to prevent diabetes insipidus in patients with LCH in CNS-risk bones.[76,77] It should be noted, however, that the DAL-HX studies administered more drugs and treated patients for 12 months.

Afterward, prednisone is given for 5 days at 40 mg/m2 every 3 weeks with the vinblastine injections (also every 3 weeks).