Diagnostic surprise in an evisceration specimen

To report a rare ocular tumor discovered incidentally after evisceration of a painful blind eye.

Observational case report.

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.

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.