A case of orbital sebaceous gland carcinoma developing in an anophthalmic socket 65 years after enucleation
A 68-year-old woman presented in the Department of Ophthalmology of Tokyo University with swelling and non-bloody discharge in her right eyelid, which had developed over several months. The right eye had been enucleated at the age of three years old, due to congenital glaucoma. She did not undergo orbital implant surgery at that time. Apart from the enucleation, the patient had an unremarkable medical history, and no family history of malignancy. She had worn the original ocular prosthesis for more than 60 years without maintenance. Since one year before presentation, the patient noticed increasing difficulty in wearing the prosthesis.\nOn examination, a firm mass was palpable beneath the right upper and lower eyelids. Cilia were present in both upper and lower right eyelids. Gadolinium-enhanced magnetic resonance imaging revealed a contrast enhanced mass measuring 38 mm × 33 mm × 36 mm in the right orbit (). A clinical work-up for systemic disease, including complete blood cell count, chemistry panel, liver enzyme panel and imaging of the chest, was negative. There was no regional lymphadenopathy, and gallium scintigraphy revealed no systemic metastasis. A histopathologic examination of transcutaneous excisional biopsies of the mass revealed lobular aggregates of atypical basaloid cells, separated by fibrovascular stroma, and an inflammatory infiltration. The atypical cells had abundant clear multi-vacuolated cytoplasm, and oval nuclei with discernible nucleoli (). Tumor cells were immunoreactive for adipophilin and perilipin, which are useful markers for sebaceous gland carcinoma (). Esentreration of the right orbit was planned by performing multiple map biopsies, including the upper and lower eyelid skin, tarsus, orbicular muscle and skin, and deep tissues below the eyebrow. Within a month, the patient underwent orbital exenteration to excise the entire mass. The tumor had well defined margins and was enclosed by a capsule (). No spread to other sites were observed. Histopathologic examination of the excised tumor demonstrated a poorly differentiated sebaceous gland carcinoma with negative margins. The eye socket was reconstructed using a free rectus abdominal muscle flap and mucosal graft. The patient recovered well, and was able to wear a prosthesis after surgery. There was no evidence of recurrence of metastasis ten months after orbital exenteration.