Orbital histiocytosis with systemic involvement

Abstract

A 70-year-old male presented with orbital masses affecting the muscular cone. His past medical history was notable for diabetes mellitus, ischemic cardiopathy, sleep-apnea syndrome, and multiple serous effusions. The first biopsy specimen of affected orbital tissue revealed fibrohistiocytic infiltration resembling xanthogranuloma or Erdheim–Chester disease (ECD). An ulterior biopsy of affected orbital tissue showed lymphocyte emperipolesis with immunopositivity for CD68 and S100 but negative staining for CD1a marker, strongly suggesting Rosai–Dorfman disease (RDD). Afterward, pericardium and peritoneal effusions resulted in constrictive pericarditis and retroperitoneal fibrosis, respectively. The absence of distinctive clinical features made the diagnosis especially challenging. Attempts to control the disease using corticosteroids, radiation, orbital surgery, and interferon were unsuccessful. Aggressive treatments such as chemotherapy were not considered appropriate due to the general deterioration of our patient. Although the possibility of two concurrent diseases (e.g., systemic ECD and orbital RDD) cannot be discarded, we interpreted the orbital findings as likely due to RDD, and the entire condition of our patient as an extranodal RDD with atypical clinicopathological findings and outcome.

Rosai–Dorfman Disease Presenting With Diplopia

“…Similar to our case, however, is the involvement of the periorbital region, not the eye itself. In a study of 7 patients, most presented with proptosis, unilateral or bilateral—a common symptom caused by a mass behind the eyes (4). As in our case, use of corticosteroid treatment failed to provide significant improvement in pain symptoms (1).…”

“…As in our case, use of corticosteroid treatment failed to provide significant improvement in pain symptoms (1). Other cases have involved lacrimal gland or concurrent intracranial masses and highlight the importance of repeated biopsies if necessary because an initial biopsy may not be representative of the underlying pathology (4).…”