Diagnostic surprise in an evisceration specimen

Area of Science:

• Ophthalmology
• Oncology
• Pathology

Background:

• Primary ocular adenocarcinoma is a rare malignancy.
• Adenocarcinoma of the retinal pigment epithelium is exceptionally rare.
• Longstanding blind eyes may harbor occult malignancies.

Observation:

• A 67-year-old female underwent evisceration for a chronically painful, blind right eye.
• Intraoperative findings revealed unusual, deeply pigmented lumps within the ocular contents.
• Histopathology confirmed primary adenocarcinoma of the retinal pigment epithelium.

Findings:

• Extensive systemic screening, including CT scans, did not identify any other primary tumor site.
• Immunohistochemical techniques ruled out metastatic adenocarcinoma from common primary sites (lung, breast, kidney).
• The diagnosis of primary ocular adenocarcinoma was established in the absence of a detectable metastasis.

Implications:

• This case highlights the importance of routine histopathological examination of all eviscerated ocular contents.
• It emphasizes the possibility of primary ocular adenocarcinoma, particularly in longstanding blind eyes.
• Early detection and diagnosis are crucial for appropriate management, even in rare presentations.