Poorly differentiated orbital neuroendocrine carcinoma

A man in his 70s presented with painless bilateral eyelid oedema and vertical diplopia. Evaluation showed a restrictive pattern of extraocular motility testing with MRI demonstrating significant enlargement of the right superior rectus and left superior oblique muscles along with right orbital fat stranding. Subsequent right orbital biopsy revealed poorly differentiated high-grade neuroendocrine carcinoma without a systemic primary site on further diagnostic workup. The patient was treated with carboplatin and etoposide and passed away from an infection a month after diagnosis. This case along with a review of other published cases highlights the varied presentation of orbital neuroendocrine carcinomas that may mimic a broad differential of orbital processes, thus requiring careful diagnostic workup. Subsequently, additional considerations in metastatic evaluation should be based on tumour histological features.