Nodulo-Ulcerative Ocular Surface Squamous Neoplasia in 27 Patients: Clinical Presentation, Management and Outcomes

To report the clinical presentation, management, and outcomes of nodulo-ulcerative ocular surface squamous neoplasia (nuOSSN).
Retrospective interventional clinical cohort study of 27 patients from a quaternary ocular oncology center.
Twenty-seven patients were diagnosed with nuOSSN during the six-year study period. The mean age at presentation was 53 years (median, 51 years; range, 25 to 81 years). Misdiagnosis was noted in 7 (26%) patients, and the mean lag time from symptom onset to presentation at our center was 14 months (median, 6 months; range, 1 to 96 months). The bulbar conjunctiva was the commonest epicenter (n = 19, 70%), and the temporal quadrant was most commonly involved (n = 13, 48%). The mean tumor diameter and thickness were 18 mm (median, 16 mm; range, 8 to 60 mm) and 3 mm (median, 2 mm; range, 1 to 5 mm), respectively. Corneal, limbal, and scleral thinning were seen in 15 (56%), 18 (67%), and 10 (37%) patients, respectively. Peripheral corneal opacification was seen in 18 (67%), and thickening around the area of scleral thinning was seen in 12 (44%) patients. Tumors belonged to the American Joint Committee for Cancer classification T2 (n = 2, 7%), T3 (n = 19, 70%), and T4 (n = 6, 22%) categories. Primary treatment (n = 19) included topical 1% 5-fluorouracil (5FU) eye drops (n = 1, 4%), excisional biopsy (n = 4, 14%), extended enucleation (n = 11, 41%), and orbital exenteration (n = 3, 11%). At a mean follow-up of 9 months (median, 3 months; range 1 to 46 months) in 19 patients who received treatment, the outcomes included local tumor recurrence in 2 patients (11%), tumor control in 16 (84%), globe salvage in 3 (16%), and lymph node metastasis in 2 patients (11%).
nuOSSN is an aggressive form of the disease that is frequently misdiagnosed due to the presence of corneoscleral thinning. Globe salvage rates are poor owing to the advanced stage of presentation.