PURPOSE: To review the clinical presentation, histopathologic analysis, management, and ocular and systemic outcome of @entity980 affecting the ocular region. DESIGN: Retrospective, noncomparative, consecutive, interventional case series. @entity1 : Thirty-three consecutive @entity1 with primary ophthalmic involvement of @entity980 from a single tertiary care center specializing in ocular oncology. MAIN OUTCOME MEASURES: Final visual acuity, @entity532 , local recurrence, and distant @entity3 . RESULTS: The mean age at presentation was 10 years (median, 7 years; range, 1 month-68 years). At presentation, 8 @entity1 (24%) were older than age 10 years and 4 @entity1 (12%) were older than 20 years. The @entity980 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 @entity5 were present for a mean of 5 weeks and included proptosis in 10 @entity1 (30%), eyelid swelling in 7 @entity1 (21%), and @entity1736 in 6 @entity1 (18%). The initial diagnosis before referral to us included @entity980 in 8 cases (24%), @entity2024 in 5 cases (15%), orbital or @entity2688 in 5 cases (15%), @entity6436 in 4 cases (12%), and others. Using the Intergroup @entity980 Study Group staging and treatment protocols, the @entity5 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 @entity5 and enucleation for the three intraocular @entity5 . Local @entity5 recurrence was detected in 6 @entity1 (18%). Orbital exenteration was necessary for @entity5 recurrence in 2 cases (6%). Long-term visual outcome of the 28 @entity1 who maintained their globe was 20/20 to 20/40 in 11 @entity1 (39%), 20/50 to 20/100 in 5 @entity1 (18%), and 20/200 to no light perception in 12 @entity1 (43%). Regional lymph node @entity3 was detected in 2 @entity1 (6%), one at initial visit and one after therapy. Distant @entity3 occurred in 2 @entity1 (6%), one detected at initial visit and one after therapy. With mean follow-up of 8.3 years, @entity5 -related @entity204 occurred in 1 @entity1 (3%). CONCLUSIONS: @entity980 can present in the orbit, eyelid, conjunctiva, and uveal tract. After treatment, @entity5 recurrence occurs in 18%, @entity3 in 6%, and @entity204 in 3%.

Clinical spectrum of primary XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'PARTICIPANTS'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'local tumor', 'tumors'] @entity532 :: ('MESH:D003925', 'Disease') :: ['treatment complications'] @entity2688 :: ('MESH:D002481', 'Disease') :: ['preseptal cellulitis'] @entity6436 :: ('MESH:D016727', 'Disease') :: ['idiopathic orbital inflammatory pseudotumor'] @entity3 :: ('MESH:D009362', 'Disease') :: ['metastasis'] @entity2024 :: ('MESH:D003231', 'Disease') :: ['conjunctivitis'] @entity980 :: ('MESH:D012208', 'Disease') :: ['Rhabdomyosarcoma', 'rhabdomyosarcoma'] @entity1736 :: ('MESH:D001763', 'Disease') :: ['blepharoptosis'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity980 :: (MESH:D012208,Disease) :: ['ophthalmic rhabdomyosarcoma']
