title: Low-dose cyclophosphamide and interferon alfa 2a for the treatment of capillary hemangioma of the orbit.
abstract: 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.
authors.affiliation: Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, Tennessee 38163, USA. mwilson5@utmem.edu
authors.forename: Matthew W
authors.initials: MW
authors.lastname: Wilson
authors.forename: Mary Ellen
authors.initials: ME
authors.lastname: Hoehn
authors.forename: Barrett G
authors.initials: BG
authors.lastname: Haik
authors.forename: Martha
authors.initials: M
authors.lastname: Rieman
authors.forename: Ulrike
authors.initials: U
authors.lastname: Reiss
mesh_terms: 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
chemical_list: D020533:Angiogenesis Inhibitors; D018906:Antineoplastic Agents, Alkylating; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D003520:Cyclophosphamide