No abstract

For patients with metastatic or locally advanced eyelid and periocular carcinoma not amenable to surgical excision, targeted therapies have shown efficacy with better tolerability compared to cytotoxic chemotherapy. Overexpression of epithelial growth factor receptor was found in squamous cell carcinomas. Vismodegib targets the mutation in the hedgehog pathway identified in basal cell carcinoma and basal cell nevus syndrome. Targeted therapies provide a novel and potentially effective treatment alternative for patients with eyelid carcinoma not amendable for surgery, including those with metastatic, locally advanced disease, advanced age, and significant comorbidities. High cost, need for long-term treatment, and toxicity are relative limitations.

Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disease in which orbital involvement can have devastating outcomes. Through a case report and review of the ophthalmic literature, we explore orbital findings, disease progression, and treatment options. Cases of orbital involvement in Erdheim-Chester disease were identified in the ophthalmic literature with a PubMed query and review of cited references. A total of 14 publications reporting 19 separate cases that included ophthalmic examination data were identified. Patient ages ranged from 26-77 years with a mean age of 50 years. Seventy-four percent (14/19) were men. Vision progression to no light perception was found in 32% (6/19) of the patients. Reviewed cases reported a variety of medical and surgical treatment approaches, however, only 53% reported cases (10/19) demonstrated disease improvement or stabilization. Erdheim-Chester disease with orbital involvement is a devastating disease with a poor prognosis. Awareness of this entity by the ophthalmologist is important as orbital signs and symptoms may manifest early, and orbital biopsy is often crucial to the definitive diagnosis.

Merkel cell carcinoma (MCC) in the eyelid and periocular region can be treated surgically, in most cases, with preservation of the eye and reasonable visual function. Adjuvant radiation therapy, sentinel lymph node biopsy, and chemotherapy should be considered for MCC of the eyelid and periocular region, especially for larger tumors that are T2b or more advanced and lesions that present with regional nodal or distant metastasis.