title: Nodulo-Ulcerative Ocular Surface Squamous Neoplasia in 6 Patients: A Rare Presentation.
abstract: OBJECTIVE
  To describe clinical presentation and histopathological features of a nodulo-ulcerative variant of ocular surface squamous neoplasia (OSSN).
  
  
  METHODS
  Retrospective study.
  
  
  RESULTS
  Six patients were identified with a nodulo-ulcerative variant of OSSN. Mean age at presentation was 56 years. One patient was immunosuppressed because of human immunodeficiency virus infection. The initial misdiagnosis was necrotizing scleritis/sclerokeratitis (n = 4), intraepithelial neoplasia (n = 1), and chalazion (n = 1). The mean delay in diagnosis as OSSN was 6 months. The lesion involved bulbar conjunctiva in 5 patients and tarsal conjunctiva in 1 patient. The mean maximum tumor dimension was 16 mm, and the mean area of conjunctivoscleral/conjunctivotarsal necrosis was 8 mm. The primary treatment for nodulo-ulcerative OSSN included excisional biopsy (n = 2), plaque radiotherapy (n = 1), and orbital exenteration (n = 3). Tumor recurrence was noted in two patients necessitating orbital exenteration. At last follow-up, globe salvage could be achieved in only 1 case. Histopathology showed invasive conjunctival squamous cell carcinoma in all cases with lesions being well-differentiated (n = 4), moderately differentiated (n = 1), and with mucoepidermoid differentiation (n = 1). Underlying scleral/tarsal tumor infiltration was evident in all 6 (100%) cases. Intraocular tumor extension was evident in 3 cases infiltrating the iris (n = 2), ciliary body (n = 3), and choroid (n = 3). Orbital tumor extension was evident in 2 cases because of tumor recurrence. No metastasis or death was recorded at a mean follow-up of 26 months.
  
  
  CONCLUSIONS
  The nodulo-ulcerative variant of OSSN is rare and is most often misdiagnosed as necrotizing scleritis/sclerokeratitis causing a delay in the diagnosis. It is an aggressive lesion with increased occurrence of intraocular or orbital tumor extension.
authors.affiliation: *Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, India; †Ophthalmic Plastic Surgery Service, Massachusetts Eye and Ear Infirmary, Boston, MA; and ‡Cornea and Refractive Surgery Service, Massachusetts Eye and Ear Infirmary, Boston, MA.
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.forename: Suzanne K
authors.initials: SK
authors.lastname: Freitag
authors.forename: James
authors.initials: J
authors.lastname: Chodosh
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D001918:Brachytherapy; D002294:Carcinoma, Squamous Cell / Q000175:diagnosis* / Q000628:therapy; D003230:Conjunctival Neoplasms / Q000175:diagnosis* / Q000628:therapy; D003320:Corneal Ulcer / Q000175:diagnosis; D003937:Diagnosis, Differential; D005142:Eyelid Neoplasms / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D013508:Ophthalmologic Surgical Procedures; D012189:Retrospective Studies; D015423:Scleritis / Q000175:diagnosis