Small cell neuroendocrine carcinoma of the orbit presenting as an orbital abscess in a young female

Abstract

Small cell neuroendocrine carcinoma (SNEC) of the sino-nasal tract is a rare tumor with aggressive behavior. Invasion of the orbit, brain and base of skull is reported in advanced cases, however clinical presentation of SNEC, primarily as an orbital mass is distinctly rare. A 25-year-old female, presented to a local ophthalmologist with sudden protrusion of eyeball and was diagnosed as an orbital abscess which was incised and drained. She presented to us after six months of initial drainage with rapid increase in protrusion and associated eyelid swelling and was lactating at the time of presentation. The patient underwent clinico-pathological work up and was diagnosed as a primary case of orbital SNEC. She received chemotherapy, with an initial response followed by massive recurrence and subsequently succumbed to the disease after 18 months of presentation to us.The authors present a literature review and describe the challenges in diagnosis and management of a primary orbital SNEC, which has high propensity to progress, recur, invade neighboring sites, and show distant metastasis inspite of multimodal therapy. Author's recommend close follow up during disease free intervals.