One category of idiopathic orbital inflammation (IOI) displays a granu
lomatous inflammatory pattern that mimics sarcoidosis, although this h
as not been extensively addressed in most published series of IOI. We
analyzed the clinicopathologic features of patients with biopsy-proven
noninfectious granulomatous inflammation of the orbit. Review of surg
ical pathology records from January 1988 to May 1992 identified 12 pat
ients with a diagnosis of sarcoidosis or other noninfectious granuloma
tous process involving the orbit. Clinical records were reviewed, and
the patients' physicians contacted to determine if the diagnosis of sa
rcoidosis was confirmed. Five cases in which the systemic diagnosis wa
s not established despite thorough evaluation are reported here. We re
port five cases of noninfectious IOI in which sarcoidosis was suspecte
d clinically and histologically. In these, however, further systemic e
valuation at 15 to 32 months (mean 22.4) failed to reveal evidence of
systemic involvement. A. spectrum of histopathologic patterns was seen
, including nonnecrotizing foreign body type granulomas, lipogranuloma
tous inflammation, and variable sclerosis. Patients with solitary orbi
tal noncaseating granulomatous inflammation should be thoroughly evalu
ated before a diagnosis of sarcoidosis is made. Practitioners should b
e aware of the existence of granulomatous IOI not associated with syst
emic sarcoidosis as a distinct clinicopathologic entity.