Wegener’s GranulomatosisWegener’s granulomatosis is characterized by necrotizing vasculitis and granulomatous inflammation in the upper respiratory tract, lung, and kidneys. It occurs predominantly in males. Orbital involvement occurs in about 20% of the cases and is usually bilateral. [
48] It is extremely difficult to make a specific diagnosis of Wegener’s granulomatosis or even a diagnosis of necrotizing vasculitis by fine needle aspiration. Usually, extremely scant material is obtained, but histiocytes and groups of necrotic cells can sometimes be identified (Figure 10-23). These lesions frequently have extensive fibrosis, and collection of adequate material by fine needle biopsy is very difficult (Figure 10-24).
SarcoidosisSarcoidosis is a multisystem, granulomatous disease of unknown cause. It occurs more frequently in women and blacks. The lacrimal gland may be palpably enlarged in 7% of patients. Patients may be considered to have a lacrimal gland tumor (Figure 10-25). Fine needle biopsy demonstrates granulomatous inflammation without necrosis. Multinucleated giant cells are usually evident (Figure 10-26). When a fine needle aspirate reveals granulomatous inflammation on the first biopsy, another aspirate should be considered to obtain culture and special stains to rule out infectious causes. Sarcoidosis is a clinical diagnosis and cannot be made by cytologic or histologic findings alone.
Eosinophilic GranulomaEosinophilic granuloma is one of the spectrum of the diseases known as histiocytosis X, which includes Hand-Schüller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma. It is thought that proliferating Langerhans’ cells are responsible for the lesion. The orbit is most often involved by unifocal disease, eosinophilic granuloma. [
49.
50] The disease usually occurs in children and teenagers and involves bone and adjacent soft tissue (Figure 10-27 and 10-28). It is common in the superotemporal portion of the orbit. The diagnosis can be readily made by fine needle aspiration provided sampling is adequate. [
51] Langerhans’ cells are evident as histiocytes with grooved or indented nuclei. Multinucleated giant cells, eosinophils, and neutrophils are present (Figure 10-29). The diagnosis can be confirmed by electron microscopy of the aspiration specimen (Figure 10-30). Treatment usually includes curettage.
References
48.
Straatsma BR, Ocular Manifestation of Wegner’s granulomatosis. Am J. Opthalmol, 1957. 49.
Baghdassarian SA, et al. Eosinophilic granuloma of orbit. Ann Opthalmol 1977.50.
Jakobiec FA, et al. Localized eosinophilic granuloma (Langerhans' cell histiocytosis) of the orbital frontal bone. Arch Opthalmol, 1980. 51.
Layfield LJ, et al. Fine-needle aspiration cytology of histiocytosis X: a case report. Diagn Cytopathol, 1988.
