Clinical features of 21 patients with orbital Langerhans cell histiocytosis

doi:10.3877/cma.j.issn.2095-2007.2020.03.003

Abstract

Abstract:

Objective

To explore the clinical features of orbital Langerhans cell histiocytosis (LCH).

Methods

Retrospective cases series. Clinical data of 21 cases (21 eyes) patients with orbital LCH in the Orbital Research Institute of the 3rd Medical Center of the People′s Liberation Army Hospital from January 2013 to June 2015 were selected. Among of them, there were 14 males (14 eyes) and 7 females (7 eyes), aged from 1 to 51 years-old with the average age of (7.4±10.0) years-old. Age, sex, clinical presentation, lesion location, imaging findings, and histopathological results, treatment method and prognosis were recorded. The clinical data were described by using frequency and percentage.

Results

All patients mainly showed lumps at frontal part or cheek, inflammatory infiltration and protopsis. Among of them, 8 cases (8 eyes) accouting for 38.10% (8/21) were found lesions at the right orbit, 13 cases (13 eyes) accouting for 61.90% (13/21) at the left orbit, 6 cases (6 eyes) accouting for 28.57% (6/21) at the upper exterior boundary of orbit, 5 cases (5 eyes) accouting for 23.81% (5/21) at lateral wall, 9 cases (9 eyes) accouting for 42.56% (9/21) at upper wall, and 1 case (1 eye) accouting for 4.76%(1/21) at lower exterior boundary. The osteolytic bone destruction was founded by computed tomography, T1WI middle-intensity signals and T2WI middle-high-intensity mixed signals was founded by magnetic resonance imaging in 17 cases (17 eyes) accouting for 80.95% (17/21) . These signals were unevenly strengthened after the enhancement with irregular strengthened zone of tumor interior. Typical LCH with hemorrhage was found by histopathological examination. 20 cases (20 eyes) were showed tumor cytoplasm and positive cytomembrane after S-100 dyeing. Only one case (one eye) was showed Ki-67 antigen positive rate accouting for 4.76% (1/21). 21 cases (21 eyes) were treated by tumorectomy. The excision tumors had uneven size and shapes without fibroid diolame. After 3 months to 1 year follow-up visits, only one case accouting for 4.76% (1/21) showed recurrence.

Conclusions

Orbital LCH was manifested by orbital lumps or exophthalmos. Imaging examination was an important diagnosis method, and accurate diagnosis was based on immunohistochemical examination. Excision was the first choice in the treatment. Patients with orbital single-system LCH have good prognosis, but follow-up analysis is needed.

Key words: Orbit, Histiocytosis, Langerhans cells, Diagnosis, Prognosis

Ronghai Pan, Rui Ma, Xinji Yang, Jianmin Ma. Clinical features of 21 patients with orbital Langerhans cell histiocytosis[J]. Chinese Journal of Ophthalmologic Medicine(Electronic Edition), 2020, 10(03): 141-146.

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