LAUSR

BACKGROUND Primary sebaceous carcinoma of lacrimal gland (SCLG) is extremely rare. Due to its clinical characteristics, it is often misdiagnosed. It is known to be aggressive so delay in diagnosis can increase the chance of local recurrence and metastasis. We report a case of primary SCLG. CASE SUMMARY A 75-year-old man was referred with a painful swelling in the right eyelid. Computed tomography scan demonstrates a 2.6 cm mass in upper outer quadrant of right orbit. After the incisional biopsy of the mass, he was diagnosed with squamous cell carcinoma of lacrimal gland. A right orbital exenteration, cervical lymph node dissection was performed. There was no involvement of the overlying periorbital skin, eyelid, or conjunctiva. Histological examination of the exenteration specimen showed tumor arising from the lacrimal gland, which had been entirely replaced by sebaceous carcinoma with sarcomatous and squamous differentiation. Neoplastic cells which were large and contained prominent nucleoli. Focal tumor remained in the posterior resection margin. Patient underwent adjuvant radiotherapy, total dose of 56 Gy with dose-per-fraction 2 Gy to primary lesion. There has been no sign of recurrence until 2-years follow-up. CONCLUSION We report a rare case of sebaceous carcinoma arising from the lacrimal gland. Accurate diagnosis of SCLG is important for appropriate treatment and prognosis prediction.