High-frequency ultrasound of extranodal limbal Rosai-Dorfman disease: affecting the conjunctiva, sclera, and cornea.
OBJECTIVE
To correlate the clinical, high-frequency ultrasound, and pathology characteristics of an epibulbar Rosai-Dorfman tumor.


METHODS
We report a case of a steroid-resistant yellow perilimbal epibulbar tumor referred for ophthalmic oncology evaluation. It was documented by slit-lamp photography and evaluated by high-frequency ultrasound. A hematology-oncology evaluation and excisional biopsy were performed.


RESULTS
Ophthalmic examination revealed a solitary yellow perilimbal epibulbar tumor. High-frequency ultrasound imaging revealed low internal reflectivity and partial-thickness scleral and corneal invasion with no extension into the anterior segment. Primary excision was performed. Although histopathology revealed large atypical histiocytes, immunochemistry found them to be both S-100 positive and CD1a negative (diagnostic of Rosai-Dorfman disease). Hematology-oncology evaluation revealed no systemic disease or links to human herpesvirus. Local control required cryotherapy and sub-Tenon steroid injection.


CONCLUSIONS
Epibulbar Rosai-Dorfman tumors can invade the sclera and are often treated by surgical excision. High-frequency ultrasound imaging should be used to determine the presence or extent of invasion before surgery.
New York Eye Cancer Center, New York, NY 10021, USA. pfinger@eyecancer.com
Paul T
PT
Finger
Henry D
HD
Perry
Sanford
S
Kempin
Codrin E
CE
Iacob
D000328:Adult; D015415:Biomarkers; D003229:Conjunctival Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D003316:Corneal Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D005260:Female; D015618:Histiocytosis, Sinus / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D015422:Scleral Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D014463:Ultrasonography
D015415:Biomarkers