Indian Journal of Pathology and Microbiology
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Year : 2023  |  Volume : 66  |  Issue : 1  |  Page : 155-158

Small cell neuroendocrine carcinoma with primary orbital involvement – The unseen, and a review of literature

1 Oculoplasty and Paediatric Ophthalmology Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India
2 Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India
3 Department of Ophthalmology, Northern Railway Central Hospital, New Delhi, India

Correspondence Address:
Deepsekhar Das
Oculoplasty and Pediatric Ophthalmology Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi –110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_1144_21

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Neuroendocrine neoplasms are derived from the epithelial lineages mainly of respiratory tract, with predominant neuroendocrine differentiation. There are only a handful of documented cases of paranasal small cell neuroendocrine carcinomas (SNEC) with primary orbital involvement. Here, the authors describe a 33-year-old male patient with rapidly progressive swelling of the right lower lid with proptosis since 4 weeks. On contrast-MRI orbit, an ill-defined multilobulated mass measuring 3.6 × 3.1 cm with intense homogenous enhancement was seen in the right retrobulbar space involving the right ethmoid sinus. On incisional biopsy, a poorly differentiated mass containing numerous small round blue cells and scanty intervening stroma with prominent necrosis and apoptosis was seen. Immunohistochemistry was strongly positive for synaptophysin. He was diagnosed as a case of SNEC and received chemotherapy, with good response till date of 9 months of follow up. The authors present a literature review and describe challenges in management of a primary orbital SNEC.

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