title: Clinical spectrum of primary ophthalmic rhabdomyosarcoma.
abstract: OBJECTIVE
  To review the clinical presentation, histopathologic analysis, management, and ocular and systemic outcome of rhabdomyosarcoma affecting the ocular region.
  
  
  METHODS
  Retrospective, noncomparative, consecutive, interventional case series.
  
  
  METHODS
  Thirty-three consecutive patients with primary ophthalmic involvement of rhabdomyosarcoma from a single tertiary care center specializing in ocular oncology.
  
  
  METHODS
  Final visual acuity, treatment complications, local recurrence, and distant metastasis.
  
  
  RESULTS
  The mean age at presentation was 10 years (median, 7 years; range, 1 month-68 years). At presentation, 8 patients (24%) were older than age 10 years and 4 patients (12%) were older than 20 years. The rhabdomyosarcoma was primarily located in the orbit in 25 cases (76%), conjunctiva in 4 cases (12%), eyelid in 1 case (3%), and uveal tract in 3 cases (9%). Symptoms or signs related to the tumor were present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 patients (21%), and blepharoptosis in 6 patients (18%). The initial diagnosis before referral to us included rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 cases (15%), orbital or preseptal cellulitis in 5 cases (15%), idiopathic orbital inflammatory pseudotumor in 4 cases (12%), and others. Using the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols, the tumor was classified as group I in 4 cases (12%), group II in 12 cases (36%), group III in 16 cases (48%), and group IV in 1 case (3%). Treatment included surgical debulking and various regimens of chemotherapy and radiotherapy for the periocular tumors and enucleation for the three intraocular tumors. Local tumor recurrence was detected in 6 patients (18%). Orbital exenteration was necessary for tumor recurrence in 2 cases (6%). Long-term visual outcome of the 28 patients who maintained their globe was 20/20 to 20/40 in 11 patients (39%), 20/50 to 20/100 in 5 patients (18%), and 20/200 to no light perception in 12 patients (43%). Regional lymph node metastasis was detected in 2 patients (6%), one at initial visit and one after therapy. Distant metastasis occurred in 2 patients (6%), one detected at initial visit and one after therapy. With mean follow-up of 8.3 years, tumor-related death occurred in 1 patient (3%).
  
  
  CONCLUSIONS
  Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. After treatment, local tumor recurrence occurs in 18%, metastasis in 6%, and death in 3%.
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 900 Walnut Street, Philadelphia, PA 19107, USA.
authors.forename: C L
authors.initials: CL
authors.lastname: Shields
authors.forename: J A
authors.initials: JA
authors.lastname: Shields
authors.forename: S G
authors.initials: SG
authors.lastname: Honavar
authors.forename: H
authors.initials: H
authors.lastname: Demirci
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003230:Conjunctival Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D005142:Eyelid Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009918:Orbital Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D015996:Survival Rate; D014604:Uveal Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy