Purpose: To report the use of a combination of low-dose cyclophosphamide and interferon alfa 2a (IFNalpha2a) for the treatment of orbital juvenile capillary hemangioma.
Design: Retrospective case series.
Participants: Five patients with juvenile capillary hemangiomas of the orbit.
Methods: Five patients with a median age of 9 weeks presented with a rapidly enlarging orbital mass. Two patients also had involvement of the upper eyelid obstructing the visual axis. Patients underwent biopsy to confirm the diagnosis before starting combination therapy with low-dose oral cyclophosphamide (10 mg/kg per day for 3 days repeated every 2 weeks) and subcutaneous IFNalpha2a (3 million units/m2 per day once daily) for a maximum treatment time of 4 to 6 months. Patients underwent serial ophthalmic, hematologic, and neurologic evaluations.
Main outcome measure: Regression of lesions after combination therapy.
Results: Four of 5 patients had marked regression of the hemangioma by 40% to 60% with subsequent reduction in proptosis, corneal exposure, and obstruction of the visual axis. None of the 4 patients developed amblyopia. Side effects included mild neutropenia and uncomplicated infections. There was no neurologic toxicity after a median follow-up of 10 months. One patient failed to respond to treatment and required further treatment with intralesional steroid injections.
Conclusions: Combination of low-dose cyclophosphamide with IFNalpha2a for a short period of time induced early and lasting regression of orbital juvenile capillary hemangiomas with minimal side effects. This regimen may be a suitable alternative that avoids the toxicity of long-term administration of interferon only. However, the experience reported here does not show if cyclophosphamide alone may be sufficient and allow complete avoidance of interferon and its potential serious side effects, and longer follow-up is needed to determine if cyclophosphamide causes any long-term harm.