AIMS AND PURPOSE: To report a rare ocular tumor discovered incidentally after evisceration of a painful blind eye. METHODS: Observational case report. RESULTS: A 67-year-old Caucasian lady presented for elective evisceration of chronically irritable phthisical right eye. A history of bilateral retinal detachment surgery 34 years prior followed by phthisis of the right eye was noted. The patient was a smoker but otherwise fit and well. Intra-operatively, the ocular contents were felt to be unusual with hard deeply pigmented lumps being present in the eviscerated tissue. The specimen was sent for histopathology. This was reported as a primary adenocarcinoma of the retinal pigment epithelium after expert histological opinion. The possibilities of a secondary adenocarcinoma with possible primary sites as lung, breast or kidney were ruled out by immuno-histochemical techniques. The patient underwent extensive systemic screening including a whole body CT scan. Exhaustive investigations have not found any other tumor site. CONCLUSION: In the absence of a recognizable source of metastasis, a diagnosis of primary ocular adenocarcinoma has been made with retinal pigment epithelium being the possible site of origin. Literature review shows that primary ocular adenocarcinoma arising from neuroepithelium is a rare but recognized occurrence especially in longstanding blind eyes. This case highlights the importance of routine histopathological examination of eviscerated ocular contents, especially in long standing blind eyes.
AIMS AND PURPOSE: To report a rare ocular tumor discovered incidentally after evisceration of a painful blind eye. METHODS: Observational case report. RESULTS: A 67-year-old Caucasian lady presented for elective evisceration of chronically irritable phthisical right eye. A history of bilateral retinal detachment surgery 34 years prior followed by phthisis of the right eye was noted. The patient was a smoker but otherwise fit and well. Intra-operatively, the ocular contents were felt to be unusual with hard deeply pigmented lumps being present in the eviscerated tissue. The specimen was sent for histopathology. This was reported as a primary adenocarcinoma of the retinal pigment epithelium after expert histological opinion. The possibilities of a secondary adenocarcinoma with possible primary sites as lung, breast or kidney were ruled out by immuno-histochemical techniques. The patient underwent extensive systemic screening including a whole body CT scan. Exhaustive investigations have not found any other tumor site. CONCLUSION: In the absence of a recognizable source of metastasis, a diagnosis of primary ocular adenocarcinoma has been made with retinal pigment epithelium being the possible site of origin. Literature review shows that primary ocular adenocarcinoma arising from neuroepithelium is a rare but recognized occurrence especially in longstanding blind eyes. This case highlights the importance of routine histopathological examination of eviscerated ocular contents, especially in long standing blind eyes.