Introduction: Orbital metastases from Neuroendocrine tumours (NET) are reported to be very rare, and can be the primary presentation or become evident up to 20 years after diagnosis. We report a series presented in the NET MDT, where ocular metastasis occurred.
Case series: A 69-yr-old woman presented with blurred vision, pain and perception of a lump around the left eye. MRI showed a lacrimal gland mass within the orbit. Excision biopsy revealed a TTF1-positive, grade 2 NET (Ki67 index 10%). Carcinoid syndrome was absent. FDG PET revealed a right lung nodule, with associated mediastinal lymphadenopathy. Octreotide scan was negative. She was treated with Cisplatin and Etoposide based chemotherapy but this was poorly tolerated and discontinued. One year later imaging revealed progression and vertebral metastasis and she received palliative radiotherapy (8Gy).
A 90 year old man presented with proptosis and restricted movements of left eye. Imaging showed a well-defined mass in the left globe with lateral rectus involvement. He underwent an R0 resection, with complete recovery. Histology showed a grade 2 NET (Ki67-5%) highly suggestive of a GEP NET metastasis.
A 60 year old man presented with symptoms of carcinoid syndrome and was found to have a sigmoid NET with hepatic metastasis. He underwent anterior resection and debulking. Histology confirmed a well differentiated NET with Ki67 index 510%. He was treated with somatostatin analogues and radionuclide therapy. Six years later, he presented with blurring of vision and was found to have a well-defined mass involving levator palpebre and superior rectus muscles. After ophthalmic consultation excision biopsy showed de-differentiated grade 3 NET with Ki67- 30%. Progression of liver metastasis with multiple metastases elsewhere was seen on imaging. He was referred for palliative chemotherapy.
Conclusion: Orbital metastases are rare, but may be the first or late presentation of NETS. Careful co-ordination between ophthalmology and NET MDTs is recommended.
Although some recent publications advocate use of radionuclide therapy for these lesions this is not a current option in the NHS.
05 Dec 2016
UK and Ireland Neuroendocrine Tumour Society