title: Juvenile Xanthogranuloma Involving the Eye and Ocular Adnexa: Tumor Control, Visual Outcomes, and Globe Salvage in 30 Patients.
abstract: OBJECTIVE
  To report clinical features and treatment outcomes of ocular juvenile xanthogranuloma (JXG).
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  There were 32 tumors in 31 eyes of 30 patients with ocular JXG.
  
  
  METHODS
  Review of medical records.
  
  
  METHODS
  Tumor control, intraocular pressure (IOP), and visual acuity.
  
  
  RESULTS
  The mean patient age at presentation was 51 months (median, 15 months; range, 1-443 months). Eye redness (12/30, 40%) and hyphema (4/30, 13%) were the most common presenting symptoms. Cutaneous JXG was concurrently present in 3 patients (3/30, 10%), and spinal JXG was present in 1 patient (1/30, 3%). The ocular tissue affected by JXG included the iris (21/31, 68%), conjunctiva (6/31, 19%), eyelid (2/31, 6%), choroid (2/31, 6%), and orbit (1/31, 3%). Those with iris JXG presented at a median age of 13 months compared with 30 months for those with conjunctival JXG. In the iris JXG group, mean IOP was 19 mmHg (median, 18 mmHg; range, 11-30 mmHg) and hyphema was noted in 8 eyes (8/21, 38%). The iris tumor was nodular (16/21, 76%) or diffuse (5/21, 24%). Fine-needle aspiration biopsy was used in 10 cases and confirmed JXG cytologically in all cases. The iris lesion was treated with topical (18/21, 86%) and/or periocular (4/21, 19%) corticosteroids. The eyelid, conjunctiva, and orbital JXG were treated with excisional biopsy in 5 patients (5/9, 56%), topical corticosteroids in 2 patients (2/9, 22%), and observation in 2 patients (2/9, 22%). Of 28 patients with a mean follow-up of 15 months (median, 6 months; range, 1-68 months), tumor regression was achieved in all cases, without recurrence. Two patients were lost to follow-up. Upon follow-up of the iris JXG group, visual acuity was stable or improved (18/19 patients, 95%) and IOP was controlled long-term without medication (14/21 patients, 74%). No eyes were managed with enucleation.
  
  
  CONCLUSIONS
  Ocular JXG preferentially affects the iris and is often isolated without cutaneous involvement. Iris JXG responds to topical or periocular corticosteroids, often with stabilization or improvement of vision and IOP.
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Wasim A
authors.initials: WA
authors.lastname: Samara
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Chloe T L
authors.initials: CT
authors.lastname: Khoo
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Emil Anthony T
authors.initials: EA
authors.lastname: Say
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Jarin
authors.initials: J
authors.lastname: Saktanasate
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Ralph C
authors.initials: RC
authors.lastname: Eagle
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address: carolshields@gmail.com.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005128:Eye Diseases / Q000175:diagnosis* / Q000628:therapy; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007429:Intraocular Pressure / Q000502:physiology; D012189:Retrospective Studies; D012871:Skin Diseases / Q000175:diagnosis / Q000188:drug therapy; D014792:Visual Acuity / Q000502:physiology*; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis* / Q000628:therapy; D055815:Young Adult
chemical_list: D005938:Glucocorticoids