Low-dose cyclophosphamide and interferon alfa 2a for the treatment of capillary hemangioma of the orbit.
OBJECTIVE
To report the use of a combination of low-dose cyclophosphamide and interferon alfa 2a (IFNalpha2a) for the treatment of orbital juvenile capillary hemangioma.


METHODS
Retrospective case series.


METHODS
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.


METHODS
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.
Wilson
Matthew W
MW
Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, Tennessee 38163, USA. mwilson5@utmem.edu
Hoehn
Mary Ellen
ME
Haik
Barrett G
BG
Rieman
Martha
M
Reiss
Ulrike
U
D020533:Angiogenesis Inhibitors / Q000008:administration & dosage*; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage*; D001706:Biopsy; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D005865:Gestational Age; D018324:Hemangioma, Capillary / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000008:administration & dosage*; D009918:Orbital Neoplasms / Q000188:drug therapy* / Q000473:pathology; D011994:Recombinant Proteins; D012189:Retrospective Studies
D020533:Angiogenesis Inhibitors; D018906:Antineoplastic Agents, Alkylating; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D003520:Cyclophosphamide