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INTRODUCTION

Carcinoid tumour is a neuroendocrine tumour with a well differentiated morphology, relatively low malig-nant potential, but frequently unpredictable clinical course. These tumours arise from neuroendocrine cells native to the primary site and most commonly affect the small intestine (67.5%) and bronchopulmo-nary system (25.3%).1

Orbital carcinoid are very rare and although primary carcinoid tumour of the orbit have been described,2 it usually presents as a metastases from ileum, colon, bronchus, mediastinum and breast.2,3,4

We present two interesting cases of orbital carci-noid metastasis (OCM) with diverse presentations and discuss the role of non-conventional imaging such as an Indium-Octreotide scan in management of these patients.

CASE REPORT

Case 1

A 46-year-old Caucasian female was referred with an incidental MRI Scan finding of an enlarged right lateral rectus muscle (Figure 1a), whilst being investi-gated for 8-month history of worsening headaches. On examination, she had 6/6 vision in both eyes, 2 mm of right proptosis with limited abduction. Computed tomography revealed a well defined mass in right orbit centred on lateral rectus muscle (Figure 1b). Orbital biopsy revealed a tumour of neuroendocrine origin with positive immunoreactivity to CAM5.2, CD56 and synaptophysin. She underwent further investigations to locate the primary tumour. Her chromogranin A level was 4.8 nmol/L and carcino-embryonic antigen level was high (6 microgram/L).

Orbit, 31(6), 379–382, 2012© 2012 Informa Healthcare USA, Inc.ISSN: 0167-6830 print/1744-5108 onlineDOI: 10.3109/01676830.2012.711890

Received 31 January 2012; revised 18 May 2012; accepted 09 July 2012

Address for Correspondence: Purnima Mehta, Consultant Ophthalmologist, University Hospital, University Hospitals of Coventry & Warwickshire NHS Trust, Clifford Bridge Road, Coventry CV2 2DX, Tel: 024 76966506, E-mail: purnima.mehta2@uhcw.nhs.uk

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Orbital Carcinoid Metastasis: Diverse Presentations and Value of

Indium-Octreotide ImagingPurnima Mehta1, Sarmi Malik1, Oludolapo Adesanya2, David Snead3, and

Harpreet Ahluwalia1

1Department of Ophthalmology, University Hospital, University Hospitals of Coventry & Warwickshire NHS Trust, Coventry CV2 2DX, 2Department of Radiology, University Hospital, University Hospitals of Coventry & Warwickshire NHS Trust, Coventry CV2 2DX, and 3Department of Cellular Pathology, University Hospital, University Hospitals of

Coventry & Warwickshire NHS Trust, Coventry CV2 2DX

ABSTRACT

Purpose: To report two cases of orbital carcinoid metastasis (OCM) with diverse presentations and the role of Indium-Octreotide scan in management of these patients.

Methods: Clinical, histological and radiological findings and management of the two patients are described. Results are presented.

Conclusions: We wish to highlight the diversity of presentations in orbital carcinoid. One patient presented with headache, while the other presented with recurrent transient ptosis. To the best of our knowledge, there have been no previous reports of OCM presenting as transient ptosis. The value of Indium-Octreotide scan in detecting the true extent of systemic disease and reducing the need for biopsy in carcinoid tumour is discussed.

KEYWORDS: Orbital carcinoid metastasis, Indium octreotide scan, Ptosis

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Whole body imaging did not reveal a primary tumour, however PET scanning and laterIndium-Octreotide scanrevealed widespread bony metastatic disease (Figure 1c), for which she had palliative radiotherapy. The patient did not have any active intervention for the OCM and continues to remain under observation for 3years with no worsening of the OCM. She developed post-operative sixth-nerve palsy following orbital biopsy which has improved significantly and now uses occlusive contact lens to control residual diplopia.

Case 2

A 54-year-old Caucasian male was referred with his-tory of sudden onset left ptosis. He had two similar untreated episodes over the last one year, with sudden onset droopy left lid resolving spontaneously after few days. His medical history was notable for ileal carcinoid tumour diagnosed a year ago with hepatic metastasis and systemic carcinoid syndrome for which he had undergone surgical excision of the tumour and treated with Lanreotide to control the symptoms.

On examination he had left complete ptosis with swelling and erythema of upper lid (Figure 2a), 4 mm of left proptosis and limited ocular motility in all direc-tions of gaze. His vision was 6/7.5 in both eyes with normal optic nerve function and appearance. Computed tomography revealed a well defined,moderately enhancing mass along medial border of levator-superior rectus complex and involving the muscle (Figure 2b).

Indium-Octreotidescan showed an abnormally high uptake in liver and left orbit suggestive of OCM (Figures 2c, 2d). The patient was managed conserva-tively with complete resolution of his ptosis and pro-ptosis in 6 weeks (Figure 2e).

Comment

This case series highlights the variability of presentation in orbital carcinoid. One patient presented with head-ache, while the other presented with recurrent transient ptosis. The largest series of OCM included 13 patients.4 The commonest presentation was mass noted by 85% of patients, 62% had diplopia, 23% had reduced vision and 15% had orbital pain. The authors reported 5-year survival of 72% and 10-year survival of 38% for patients with OCM.4

To the best of our knowledge, recurrent transient ptosis as a presenting symptom has not been described before. The ptosis in the second patient occurred three times and resolved spontaneously on all occasions. Knox et al. reported 2 adult patients with OCM who presented with unilateral acute, recurrent orbital and ocular adnexal inflammation which resolved sponta-neously in 2 weeks.5 The authors suggested that the presentation could be due to spontaneous release of inflammatory mediators intrinsic to the OCM from mechanical disturbance of the orbital tumour such as eyelid rubbing.It is possible that our second patient may

FIGURE 1 (1a and 1b): Coronal MRI Scan (small arrow) and Axial CT scan (long arrow) showing a well-defined mass in right orbit cen-tred on lateral rectus muscle (1c) Indium-Octreotide scan showing increased uptake in right orbit (small black arrow) and extensive bony metastasis involving the dorsal, lumbar and cervical spine as well as the right humerus, and both pelvis and femur (long black arrow).

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have had the recurrent ptosis, swelling and erythema of the upper lid due to release of inflammatory mediators.

Conventional staging imaging techniques, such as computerized tomography (CT) and magnetic reso-nance (MRI) imaging, have traditionally been used to identify and further characterize carcinoid tumours. However, localizing metastasis may often be difficult using these imaging techniques.Increasingly radio-pharmaceutical-labelled imaging techniques such as MIBG scans and Octreotide scans are being widely applied to localize primary and/or metastatic carcinoid tumours. The application of imaging with such pep-tides has detected previously clinical occult metastases in patients with carcinoid tumours and has been used to identify patients suitable for treatment with these radiopharmaceuticals.6

Octreotide scan, which is radionuclide scanning fol-lowing the injection of 111 Indium radiolabelled octreo-tide (somatostatin analogue) is a method of localizing somatostatin positive tumours such as carcinoid tumour (which express somatostatin receptors).7 This scan is able to detect carcinoid tumours that bind octreotide and are larger than 1–1.5 cm.8

MIBG scan, is scintigraphy following injection with Iodine-131-Metaiodobenzylguanidine [123I]MIBG.6 Metastatic orbital carcinoid tumours have been reported to have increased [123I]MIBG uptakeattributed to the ele-vated serotonin levels in these tumours.9 Radiolabelled octreotide, in general detects more metastatic lesions than [123I]MIBG in patients with neuroendocrine tumors.6 MIBG scan was not performed in either of our patients.

Isidori et al.10 evaluated the use of 123I-MIBG and 111Indium Octreotide scanning in 40 cases of neuroen-docrine tumours including 6 patients (15%) with ocular metastasis (5 choroidal lesions and 1 orbital lesion). Three out of 4who were screened with 111Indium Octreotide showed positive uptake but none of the 6 patients showed positive uptake with the 123I-MIBG. The authors concluded scintigraphy with radiolabelled octreotide was useful in identifying orbital/ocular metastasis even if unsuspected, thereby allowing earlier intervention.

Indium-Octreotide scan in the second patient showed increased uptake in the left orbit, which was highly suggestive of a carcinoid tumour, thereby avoid-ing the need for an orbital biopsy. In the first patient it

FIGURE 2 (2a) Clinical photo showing left ptosis and lid swelling at the time of presentation; (2b) Sagittal CT reformat of left orbit with contrast showing amoderately enhancing well defined mass running along the medial border of the levator-superior rectus complex and involving the muscle (white arrow). (2c) SPECT (Single photon emission computerized tomography) radionuclide Indium-Octreotide scan demonstrating mildly increased uptake in the left orbital mass; (2d) Planar (anterior and posterior) radionuclide Indium-Octreotide scan demonstrating a neuroendocrine deposit in the liver; (2e) Clinical photo showing complete resolution of left ptosis after 6 weeks.

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helped in identifying the bony metastasis that was not seen in conventional CT scan. The first patient is being observed for OCM and the second is currently awaiting somatostatin receptor-targeted radionuclide therapy.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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2. Zimmerman LE, Stangl R, Riddle PJ. Primary carcinoid tumor of the orbit. A clinicopathologic study with histochemical and electron microscopic observations. Arch Ophthalmol 1983;101:1395–1398.

3. Shetlar DJ, Font RL, Ordonez N, et al. A clinicopathologic study of three carcinoid tumors metastatic to the orbit. Immunohistochemical, ultrastructural, and DNA flow cyto-metric studies. Ophthalmology 1990;97:257–264.

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5. Knox RJ, Gigantelli JW, Arthurs BP. Recurrent orbital inflam-mation from metastatic orbital carcinoid tumor. Ophth Plast Recon Surg 2001;17:137–139.

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9. Hanson MW, Schneider AM, Enterline DS, Feldman JM, Gockerman JP. Iodine-131-metaiodobenzylguanidine uptake in metastatic carcinoid tumor to the orbit. J Nucl Med1998; 39:647–650.

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