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.
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 humanherpesvirus. 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.
Authors: Franz Fogt; Thomas Rüediger; Albert J Augustin; Dale M Frank; Andreas Rosenwald; Axel Wellmann; Vivian Lee Journal: Ocul Oncol Pathol Date: 2019-04-10