Sebaceous Carcinoma, Concern for Muir-Torre Syndrome
Our patient presented with a greater than one year history of a non-resolving right eyelid mass that began to hemorrhage due to accidental trauma. Exam was remarkable for and extensive multi-lobar ulcerated mass found along the right lower lid along with two other skin lesions on the right brow and left cheek. Neuro-imaging demonstrated a large mass that extended into the zygoma and the conjunctiva with no clear plane between the mass and the cornea. Biopsy of the facial lesions revealed sebaceous carcinoma of the right eyelid and left cheek, and squamous carcinoma in situ of the right brow, with microsatellite instability on immunohistochemistry, concerning for Muir-Torre syndrome. Neoadjuvant chemotherapy to reduce tumor burden along with PET scan with surgical resection and sentinel lymph node biopsy was recommended to stage and treat the tumor, however patient had refused the PET scan and systemic testing. He was lost to follow up for 2 months and returned with worsening tumor burden. CT scan of his body revealed scattered bilateral pulmonary nodules, concerning for metastatic disease. Surgical resection at the time would have required extensive reconstruction which the patient did not want. With many multi-disciplinary and patient discussions, it was decided to start neoadjuvant chemotherapy in hopes of reducing the tumor burden, although there is limited evidence in the literature on the efficacy of doing so in the setting of likely metastatic disease.
Presentation Date: 01/20/2022
Issue Date: 02/11/2022
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