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Endocrine

pp 1–9 | Cite as

Orbital metastases from neuroendocrine neoplasms: clinical implications and outcomes

  • Lukasz Kamieniarz
  • Eleni ArmeniEmail author
  • Luke Furtado O’Mahony
  • Charlotte Leigh
  • Lukon Miah
  • Akshay Narayan
  • Ankit Bhatt
  • Nicholas Cox
  • Dalvinder Mandair
  • Shaunak Navalkissoor
  • Martyn Caplin
  • Christos Toumpanakis
Original Article
  • 81 Downloads

Abstract

Purpose

Neuroendocrine neoplasms (NENs) may rarely metastasise to the orbit. Published data on epidemiology, incidence and preferred treatment is limited. We present the largest cohort of symptomatic and asymptomatic NEN patients with orbital metastases and data on epidemiological parameters, symptoms as well as diagnostic/treatment modalities used.

Methods

We identified patients from our internal NEN database of patients who had also undergone Gallium68-DOTATATE PET (Ga68-DOTA). The diagnosis of orbital metastatic NEN was made on somatostatin receptor imaging and confirmed on a dedicated MRI of orbits.

Results

We identified 27 patients of 994 patients evaluated with Ga68-DOTATATE PET imaging during their surveillance monitoring in our department; 15 female, average age at NEN diagnosis 53 years and orbital metastatic NEN diagnosis 59 years. The majority of NEN primaries originated from small bowel (18/27, 66.4%) or pancreas 4/27 (4/27, 14.8%). Hepatic with or without concomitant skeletal metastases were present in 23/27 (85%) of patients. Ocular symptoms and/or signs were evident in 11/27 (41%) of patients. 5/11 symptomatic patients underwent external beam radiotherapy (EBRT) resulting in complete symptoms resolution. The 5-year survival was estimated at 84.1%.

Conclusions

Orbital metastases of NEN have a relatively low prevalence, more commonly associated with small bowel primary. Extraocular muscles are primarily affected, irrespectively of liver disease burden. Survival does not seem to be affected. EBRT is an efficacious treatment modality for both symptom relief and tumour growth control. Administration of peptide receptor radionuclide therapy may occasionally induce temporary ocular symptoms, which resolve following treatment with a short course of steroids.

Keywords

Neuroendocrine tumour Carcinoid Orbital neoplasm Orbital metastasis Rare metastases Orbit 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

12020_2019_2130_MOESM1_ESM.docx (16 kb)
Supplementary Information

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Lukasz Kamieniarz
    • 1
  • Eleni Armeni
    • 2
    Email author
  • Luke Furtado O’Mahony
    • 1
  • Charlotte Leigh
    • 1
  • Lukon Miah
    • 1
  • Akshay Narayan
    • 1
  • Ankit Bhatt
    • 1
  • Nicholas Cox
    • 1
  • Dalvinder Mandair
    • 2
  • Shaunak Navalkissoor
    • 3
  • Martyn Caplin
    • 2
  • Christos Toumpanakis
    • 2
  1. 1.UCL Medical SchoolLondonUK
  2. 2.Neuroendocrine Tumour Unit, ENETS Centre of ExcellenceRoyal Free HospitalLondonUK
  3. 3.Nuclear Medicine DepartmentRoyal Free HospitalLondonUK

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