Paraneoplastic sarcoid-like reactions and the eye

Abstract

Background: Sarcoid-like reactions have been reported and confirmed by histopathology in patients with malignant disease. This series demonstrates the complex relationship of malignancy and sarcoidosis as pertaining to the eye, which, to the best of our knowledge, has not been previously reported in the literature.

Methods: Retrospective case study of five patients with sarcoid-like reactions. Patients 1 to 4 represent patients with ocular sarcoid-like reaction and systemic malignant disease. Patient 5 had ocular malignancy and systemic sarcoid-like reaction; workup revealed renal cell cancer. For each patient, other etiologies of nonnecrotizing granulomatous inflammation were excluded.

Results: Sarcoid-like reactions have been described in the literature when nonnecrotizing granulomas occur in association with malignancy and in the absence of multiorgan involvement as seen with systemic sarcoid. In our series, sarcoid-like reactions involved the vitreous in three patients, retina in one patient, and the choroid and lung in one patient. Sarcoid-like reaction preceded the diagnosis of malignancy in two patients, was found concomitantly with malignancy in one patient, and followed malignancy in two patients. Two patients had hematologic malignancy, one patient had endometrial carcinoma, one had renal cell carcinoma, and one patient had both renal cell carcinoma and uveal melanoma. Four patients had findings of nonnecrotizing granulomas confirmed by histopathology.

Conclusion: Sarcoid-like reactions can occur in the eye, and ocular malignancies may incite sarcoid-like reaction. Ocular sarcoid-like reactions have paraneoplastic features in that they can occur at a site distant from malignancy and may precede, occur simultaneously with, or follow malignancy.