Table 1.
Clinical and histologic features of our 5 patients with LCH.
| Case | Age | Sex | Race | History | Orbit | Site | Size [mm] |
Bone Erosion | Eye Exam | MF [per HPF] |
S100 | CD1a | Systemic disease | TEM | Follow-up | Therapy |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| # 1 | 8 yrs | ♂ | AA | trauma (orbital contusion with progressive swelling) | superotemporal (lacrimal gland area) | OS | 20x15 | + | VA 20/20; limitation of gaze superotemporally (OS) | 0 | + | N/A | no | BG | N/A | N/A |
| # 2 | 3 yrs | ♀ | C | progressive eyelid swelling after trauma (minor fall) | superior, preseptal lesion | OD | + | full extraocular muscle movement | 0 | N/A | N/A | no | N/A | 11 yrs w/o recurrence | etoposide (VP-16; 168 mg i.v. per week) over 12 weeks | |
| # 3 | 1 yrs | ♂ | C | orbital trauma (prior to complaints) | anterior | OS | N/A | + | ptosis | 1 | N/A | N/A | bony erosions leg, skull | N/A | 3 yrs w/o recurrence | etoposide (VP-16; 100mg i.v.) over 10 weeks + prednisone (5mg) |
| # 4 | 8 mo | ♂ | HA | right facial swelling, pretreatment with antibiotics | inferolateral | OD | 14×19×18 | + | VA 20/20; full extraocular muscle movement / no strabism | 0 | + | + | no | N/A | 2 yrs w/o recurrence | low dose methotrexate + prednisone (5 mg, bid) over 1 year |
| # 5 | 9 yrs | ♀ | AA | trauma (orbital contusion), pretreatment with topical antibiotics | superolateral | OD | 29×18×35 | + | VA 20/25; full extraocular muscle movement / no strabism | 0.1 =1 MF /10HPF | + | + | no | BG | under treatment | vinblastine (6mg/m2 i.v. per week) + 6-MP (15mg/m2 p.o. per week) + prednisone over 1 year |
AA: African-American; C: Caucasian; HA: Hispanic-American; HPF: high power field; MF: mitotic figures; mo: months; TEM: transmission electron microscopy; yrs: years; BG: Birbeck granules; wo:without; N/A: not available