title: Primary ophthalmic rhabdomyosarcoma in 33 patients.
abstract: OBJECTIVE
  To review the findings, management, and outcome in 33 cases of primary ophthalmic rhabdomyosarcoma.
  
  
  METHODS
  The records of 33 consecutive patients from a single ocular oncology center were analyzed retrospectively for outcomes of final visual acuity, local recurrence, and distant metastasis.
  
  
  RESULTS
  Rhabdomyosarcoma was primarily located in the orbit m 25 cases (76%), conjunctiva in 4 (12%), eyelid in 1 (3%), and uveal tract in 3 (9%). Findings had been present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 (21%), and blepharoptosis in 6 (18%). The initial diagnoses before referral to us included primarily rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 (15%), cellulitis in 5 (15%), and pseudotumor in 4 (12%). Tumors were classified according to the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols as group I in 4 cases (12%), group II in 12 (36%), group III in 16 (48%), and group IV in 1 case (3%). Treatment included surgical debulking, chemotherapy, and radiotherapy. Local tumor recurrence was detected in 6 patients (18%), lymph node spread in 2 (6%), and distant metastasis in 2 (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 (18%), and 20/200 or worse in 12 (43%). Mean follow-up was 8.3 years; tumor-related death occurred in 1 patients (3%).
  
  
  CONCLUSIONS
  Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. Following treatment, local tumor recurrence occurs in 18% of cases, metastasis in 6%, and death in 3%.
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, 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; D003131:Combined Modality Therapy; D003230:Conjunctival Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005142:Eyelid Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009918:Orbital Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate; D014604:Uveal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy