Abstract
Purpose :
Juvenile Xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytic disorder that occurs predominantly in infants. To the best our knowledge, there are no reports of pathological prevalence of this condition in the pediatric population. Moreover, JXG cases affecting exclusively the skin of the eyelid are extremely rare. The purpose of this study is to report the histopathological findings of cutaneous periocular JXG in this population.
Methods :
All ocular and periocular lesions in the Montreal Children's Hospital and Henry C. Witelson Ocular Pathology Laboratory databases were analyzed over a 10-year period (November 2006 to November 2016). Cases diagnosed with JXG were reviewed with respect to age, gender, localization, size, treatment and other site involvement.
Results :
Of the 7311 patients analyzed, 682 had ocular or periocular lesions (9.32%). Of these, 7 (1.02%) patients had JXG, with a mean age of diagnosis of 5.57 years old. Six (85.71%) of these patients were female and one (14.28%) was male. All specimens analyzed were unilateral and confined to the skin of the eyelid[PL1] . The most common site of involvement was the left upper eyelid (n=5; 71.42%) followed by the right upper eye lid (n=2; 28.57%). The size of the specimens ranged from 3x3x2 mm to 15x9x5 mm. There was agreement between the clinical and pathological diagnosis in only 1 case (14.28%). All patients were treated with an excision biopsy. The presence of typical histopathological features of JXG, including histiocytic granulomas and Touton multinucleated giant cells, were observed in all cases.
Conclusions :
JXG is uncommon and to the best of our knowledge, this is the largest series of exclusive eyelid involvement presenting predominantly in the upper eyelid. The lack of ocular involvement contributed to the low clinical diagnostic accuracy. It is important that clinicians and pathologists are aware of this rare presentation, therefore JXG should be considered as a differential diagnosis in solitary eyelid masses in children.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.