Clinical analysis of adult onset orbital xanthogranuloma

Abstract

Objective: To investigate the clinical, imaging and pathological features of adult onset xanthogranuloma. Methods: Retrospective case series study.The clinical data of 4 adult patients with adult onset xanthogranuloma treated in the Tianjin Medical University Eye Hospital from 2015 to 2017 were analyzed. Results: There were 1 male and 3 females, aged 46-73 years, with an average age of 55 years. The duration of eyelid swelling was from 6 months to 6 years. One case was binocular and 3 cases were monocular. There were progressive enlargement of eyelid swelling and subcutaneous cord nodules with poor mobility and no tenderness in all 5 eyes. The eyelid skin in 2 patients (2 eyes) was bulging and yellow. CT examination showed swelling of the eyelids and soft tissue thickening in 5 eyes. There were lacrimal gland enlargement in 3 eyes, upper rectus muscle involvement in 3 eyes, external rectus muscle involvement in 1 eye, lower rectus muscle involvement in 1 eye, superior oblique muscle involvement in 1 eye, and involvement of both the superior and inferior rectus muscles in 1 eye. In 1 patient (1 eye), the lesion showed low and medium signals on T(1) weighted images and T(2) weighted images. Three patients (4 eyes) underwent color Doppler ultrasonography, showing the echo zone in the irregularly shaped eyelid was strong, the internal echo was uneven, the local echo was low, and the boundary was not clear. One of the eyes showed a blood flow signal. All patients underwent surgical resection. The pathological results showed infiltration of foamy tissue cells, Touton cells, lymphocytes and plasma cells. Immunohistochemistry demonstrated 3 patients (4 eyes) expressed CD68, and 1 patient (1 eye) expressed S-100. After 6 months to 2 years of follow-up, 3 patients had no recurrence. One patient developed eye discomfort at 6 months after surgery, and the symptoms were relieved after treatment with triamcinolone. Conclusions: Adult onset xanthogranuloma mainly manifest as eyelid swelling, periorbital mass and skin color changes, generally with no systemic involvement. Imaging examination mainly shows the involvement of soft tissues, lacrimal glands and muscles around the eyelids. The main features of the pathology are infiltration of foamy tissue cells, Touton giant cells, lymphocytes and plasma cells. (Chin J Ophthalmol, 2019, 55: 381-386).