Pediatric Castleman Disease Manifesting as a Lacrimal Gland Tumor.

Castleman disease constitutes a rare spectrum of conditions with rheumatologic, hematologic, and oncologic features. Orbital involvement is extremely rare with only several cases involving the lacrimal gland. This report describes Castleman disease in a 15-year-old patient who presented with progressive left eye bulging and intermittent periorbital pain. Neuroimaging demonstrated a well-defined, enhancing, homogeneous left lacrimal gland mass with decreased diffusivity, smooth bone remodeling, and anteromedial globe displacement, and no other systemic findings. The lesion was excised in toto and histopathology revealed features of hyaline vascular-type Castleman disease. This is the first reported case of Castleman disease in a pediatric patient with an isolated lacrimal gland lesion. It emphasizes the importance of considering Castleman disease among other lymphoproliferative diseases in the differential diagnosis for patients presenting with a well-circumscribed, homogeneous orbital mass associated with decreased diffusivity and bone remodeling.