Extranodal Rosai-Dorfman of the Pancreas Presents with Bilateral Granulomatous Anterior Uveitis

Abstract

Background/aims: To report a case of a 65-year-old female who presented with bilateral anterior granulomatous uveitis, which led to the diagnosis of Rosai-Dorfman Disease (RDD) and the discovery of an associated pancreatic mass.

Methods: This is a retrospective case report of a single patient.

Results: A 65-year-old African American Female received a full workup for bilateral anterior granulomatous uveitis and was found to have a pancreatic mass on imaging. Biopsy of this mass was diagnostic for RDD, a rare proliferative histiocytic disorder, which classically presents with painless cervical lymphadenopathy, leukocytosis, and hypergammaglobulinemia.

Conclusion: Although rare, ocular involvement, including eyelid and orbital masses, and anterior uveitis, can herald the development of other systemic manifestations or RDD and can aid in the diagnosis of this systemic disease.

Keywords: Pancreatic mass; Rosai-dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Uveitis.

Fig. 1

Clinical photographs and imaging from case. a, b Slit lamp photographs of the patient at presentation showing diffuse circumferential keratic precipitates (a) and diffuse conjunctival injection with ciliary flush (b). c MRI abdomen, T-1 sequence showing hypoenhancing lesion of the pancreatic tail, suspicious for pancreatic adenocarcinoma. d Gross specimen of resected pancreatic mass measuring 2.1 × 1.7 × 1.1 cm.

Fig. 2

Histology with immunohistochemistry. a Histology shows abundant histiocytes with foamy eosinophilic cytoplasm and interspersed chronic inflammatory cells. b Demonstration of emperipolesis, the characteristic feature of RDD in which histiocytes contain phagocytosed lymphocytes. c, d Immunohistochemical stains positive for CD163 (c) and S100 (d), consistent with RDD. All photos hematoxylin and eosin; 20× magnification.