Mittal Ruchi, Kaza Hrishikesh, Agarwal Sunil, Rath Suryasnata, Gowrishankar Swarnalatha
Dalmia Ophthalmic Pathology Service, LV Prasad Eye Institute, Mithu Tulsi Chanrai Campus, Bhubaneswar, India.
Ophthalmic Plastic Surgery, Orbit and Ocular Oncology Services, LV Prasad Eye Institute, Mithu Tulsi Chanrai Campus, Bhubaneswar, India.
Saudi J Ophthalmol. 2019 Jul-Sep;33(3):308-311. doi: 10.1016/j.sjopt.2018.07.003. Epub 2018 Jul 27.
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.
鼻窦小细胞神经内分泌癌（SNEC）是一种具有侵袭性的罕见肿瘤。晚期病例有侵犯眼眶、脑和颅底的报道，然而，SNEC主要表现为眼眶肿块的临床症状极为罕见。一名25岁女性因眼球突然突出就诊于当地眼科医生，被诊断为眼眶脓肿并进行了切开引流。初次引流6个月后，她因眼球突出迅速加重及伴有眼睑肿胀前来我院就诊，就诊时正在哺乳期。患者接受了临床病理检查，被诊断为原发性眼眶SNEC。她接受了化疗，起初有反应，但随后大量复发，在就诊于我院18个月后最终死于该病。作者进行了文献综述，并描述了原发性眼眶SNEC诊断和治疗中的挑战，尽管采用了多模式治疗，该肿瘤仍有很高的进展、复发、侵犯邻近部位及远处转移倾向。作者建议在无病间期密切随访。
