Nodulo-Ulcerative Ocular Surface Squamous Neoplasia in 6 Patients: A Rare Presentation

Purpose 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.