Poorly differentiated orbital neuroendocrine carcinoma

Abstract

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.

Diagnostic and Therapeutic Management of Primary Orbital Neuroendocrine Tumors (NETs): Systematic Literature Review and Clinical Case Presentation

“…Surgery represented the backbone of treatment in twelve patients, of whom five also received postoperative radiotherapy and two received chemotherapy. In one case, it was proposed that CT and RT treatment be combined [18], while two patients, including ours, were treated with exclusive medical treatment [30]. No data about the therapeutic algorithm proposed was available in one report.…”

“…When using functional imaging techniques in the diagnostic work-up of orbital NENs, we must consider that somatostatin receptors are expressed in several normal human tissues, including the brain [94][95][96], and that (F-18) FDG is accumulated in the brain with a very high uptake. Despite the use of PET/CT, as recommended by European guidelines from 2017 [83], only three patients, including our case [18,30], underwent functional studies, and only two underwent double-tracer PET/CT.…”