High-frequency ultrasound of extranodal limbal Rosai-Dorfman disease: affecting the conjunctiva, sclera, and cornea

Cornea. 2007 Aug;26(7):888-90. doi: 10.1097/ICO.0b013e318074e439.


Purpose: 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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biomarkers
  • Conjunctival Diseases / diagnostic imaging*
  • Conjunctival Diseases / pathology
  • Corneal Diseases / diagnostic imaging*
  • Corneal Diseases / pathology
  • Female
  • Histiocytosis, Sinus / diagnostic imaging*
  • Histiocytosis, Sinus / pathology
  • Humans
  • Scleral Diseases / diagnostic imaging*
  • Scleral Diseases / pathology
  • Ultrasonography


  • Biomarkers