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Year : 2014  |  Volume : 6  |  Issue : 2  |  Page : 66-68

Unusual presentation of metastatic neuroendocrine (carcinoid) tumor in the orbit

Department of Oculoplasty, Sydney Eye Hospital, 8, Macquarie Street, NSW-2000, Australia

Date of Web Publication6-Feb-2015

Correspondence Address:
Manju Meena
Department of Oculoplasty, Sydney Eye Hospital, 8, Macquarie Street, NSW-2000
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DOI: 10.4103/1858-540X.150998

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Purpose: To report a rare presentation of metastatic neuroendocrine tumor in the orbit. Case report: A 43-year-old female presented with a left painful proptosis of 4 days duration associated with nausea and vomiting. There was a history of multiple liver metastases from an unknown primary carcinoid tumor for 7 years. Ocular examination revealed severe eyelid swelling associated with conjunctival injection, chemosis and complete external ophthalmoplegia. The visual acuity was 20/20 in right eye and no light perception in left eye. The initial clinical diagnosis was orbital cellulitis with optic nerve compression. Orbital imaging showed gross enlargement of left lateral rectus muscle with optic nerve compression consistent with a carcinoid metastasis. Surgical resection of the tumor mass along with the involved muscle was performed. Histopathology confirmed the diagnosis of neuroendocrine (carcinoid) tumor infiltrating fibro fatty tissue and skeletal muscle with widespread necrosis. On Immunohistochemical staining the tumor cells were positive for chromogranin A, synaptophysin and cytokeratin. Post-operative external beam radiotherapy (EBRT) and systemic chemotherapy were given. Conclusion: Acute necrosis in metastatic orbital carcinoid tumor can lead to orbital inflammation which could mimic orbital cellulitis. Systemic history and orbital imaging play an important role in differentiating the infective etiology from metastasis.

Keywords: Carcinoid, chromogranin, histopathology, neuroendocrine, synaptophysin

How to cite this article:
Meena M, Reddie IC, Kourt G, Martin PA. Unusual presentation of metastatic neuroendocrine (carcinoid) tumor in the orbit. Sudanese J Ophthalmol 2014;6:66-8

How to cite this URL:
Meena M, Reddie IC, Kourt G, Martin PA. Unusual presentation of metastatic neuroendocrine (carcinoid) tumor in the orbit. Sudanese J Ophthalmol [serial online] 2014 [cited 2021 Sep 19];6:66-8. Available from: https://www.sjopthal.net/text.asp?2014/6/2/66/150998

  Introduction Top

Carcinoid tumors are neuroendocrine tumors which are slow growing, low-grade neoplasms derived from enterochromaffin cells, which are found primarily in the gastrointestinal tract and bronchial tree. [1],[2] It can rarely originate in the ovary, thymus, parotid gland, breast, and testis. [3],[4] Liver metastasis is the most common presentation of distant disease which can lead to the carcinoid syndrome. Carcinoid tumor metastasis to the orbit accounts for only 4% to 5% of orbital metastatic disease. [5] It typically present as a unilateral orbital mass inducing proptosis and diplopia. [6] Orbital cellulitis is an unusual presentation and sparsely reported. We report a patient with widely metastatic carcinoid tumor and an orbital mass who clinically presented as acute orbital inflammation.

  Case report Top

A 43-year-old woman was referred to our hospital for the management of left orbital cellulitis. There was a history of acute onset left painful proptosis of 4 days duration. She also noted severe nausea and vomiting associated with left periorbital swelling. She denied antecedent trauma and illness. The patient was treated with intravenous antibiotics (Ceftriaxone 2 g daily) before referral. Seven years earlier she was diagnosed with liver metastasis from an unknown primary neuroendocrine(carcinoid) tumor. Despite systemic chemotherapy and local radiotherapy her tumor progressed with increase in size and number of metastases. There were frequent episodes of flushing, abdominal cramps, diarrhoea associated with elevated urinary 5-Hydroxyindoleacetic acid (HIAA). The patient was also known to have thalassemia minor.

On ocular examination her visual acuity was 20/20 in right eye and no light perception in left eye. A left relative afferent pupillary defect and 5 mm of left proptosis on exophthalometry were noted [Figure 1]. The ocular movements were restricted in all position of gaze and there was resistance to retropulsion. There was severe conjunctival injection and chemosis. The patient was afebrile and normotensive. A computed tomography scan (with contrast) of the orbits showed proptosis of the left globe with a fusiform-shaped grossly enlarged left lateral rectus muscle. There was a diffuse infiltrating isodense mass in the lateral rectus muscle measuring 3 × 2 × 1.5 cm 3 . There was medial displacement and compression of optic nerve by the enlarged muscle [[Figure 2]a,b]. Subsequent magnetic resonance imaging was undertaken with Gadolinum contrast. This correlated with the CT images, also suggesting recent hemorrhage within the lesion.
Figure 1: Clinical photograph showing left proptosis with periorbital edema

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Figure 2: (a) A contrast enhanced axial CT scan of the orbits illustrating left proptosis, with fusiform-shaped enlarged left lateral rectus muscle. There is an ill-defined isodense lesion infiltrating the muscle and displacing the optic nerve medially. (b) Coronal CT scan image showing compression of the left optic nerve by the enlarged lateral rectus muscle

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She underwent left lateral orbitotomy following an endoscopic medial wall decompression. The tumor was excised completely along with lateral rectus muscle. Microscopic examination showed a circumscribed metastatic tumor infiltrating fibro fatty tissue and skeletal muscle, with widespread necrosis. The immunohistochemistry findings were consistent with neuroendocrine tumor with positive staining for Chromogranin A, Synaptophysin, cytokeratin (CAM 5.2, AE1/3), CD56 and Neuron specific enolase (NSE) [[Figure 3]a-d].
Figure 3: Histopathology section from tumor show (a) Sheet of tumor cells, ovoid nuclei, finely specked chromatin pattern. The cytoplasm was acidophilic with poorly defined cell margins. (hematoxylin-eosin, ×400 ). (b) Strong cytoplasmic synaptophysin immunostaining favoring neuroendocrine differentiation (×400), and (c) Marked cytoplasmic chromogranin immunostaining (×400). (d) Tumor cells show positivity for cytokeratin CAM 5.2 (×400)

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The post-operative period was uneventful. Despite the systemic chemotherapy and EBRT to the orbit and liver she developed further metastases in kidney, lung, thyroid, mediastinal lymph nodes. The patient died 3 years after orbital surgery.

  Discussion Top

Metastases to orbital structures are an unusual occurrence in neuroendocrine tumors. The risk of metastases has been demonstrated to increase with tumor size larger than 1 cm. [7] Reports suggest that uveal tract carcinoid metastases are more commonly noted in bronchial lesions, whereas extraocular muscle (EOM) metastases more commonly occur in gastrointestinal primary carcinoids. [8],[9],[10] The site of metastasis may therefore give some clue to the primary source if this is not known. The average reported age of presentation with orbital metastasis of the neuroendocrine tumor is 65 years. [5],[10],[11] Our patient had relatively early age of presentation.

The most common presenting symptoms of orbital carcinoid tumors are proptosis and limitation of ocular movements from a mass lesion. Orbital inflammation is a rare presentation of this group of tumors. On literature review, there were few reported cases of metastatic orbital carcinoid tumors which presented as orbital inflammation. Radiographically and histopathologically, the orbital tumor in these reports contained central necrosis. [12],[13] We describe herein a case of metastatic orbital carcinoid tumor demonstrating acute unilateral inflammation of 4 days duration with similar histopathological findings.

Carcinoid tumor is occasionally associated with "carcinoid syndrome" which is characterized by cutaneous flushing, diarrhea, hypotension, light headedness and bronchospasm. The tumor intrinsic peptides hormone and vasogenic amines mediate these symptoms. This syndrome occurs commonly in patients with hepatic metastasis and, thus, is at risk for inflammatory mediator release into the hepatic circulation.

In our case the orbital inflammation was of acute onset and was ipsilateral to the orbital metastasis. The swelling was non-responsive to systemic antibiotics which excludes the possibility of any infective etiology. Therefore, inflammation secondary to tumor necrosis remains a possibility. Furthermore, the histopathology also suggested the presence of widespread necrosis within the tumor. Our patient had a history of symptoms suggestive of carcinoid syndrome prior to presentation. However there were none at the time of presentation. We believe that spontaneous tumor necrosis in our case could have precipitated the release of the inflammatory mediators.

The optimum management of metastatic orbital carcinoids remains controversial. In the past, some patients underwent exenteration. [13] Currently, excisional biopsy is offered for small, discrete tumors and a combination of palliative 30-Gy radiotherapy and chemotherapy is administered for diffuse or larger, unresectable tumors. [10],[13] Our patient underwent complete surgical resection of the tumor followed by adjuvant radiotherapy and chemotherapy. The patient was free from orbital recurrence following tumor removal. However, she died due to further progression of widespread tumor metastasis.

Perioperative management of these patients is difficult and requires an experienced anesthetic team and the availability of intensive care facilities. The tumor cells secretes vasoactive substances which can cause extreme autonomic disturbance including labile blood pressure and temperature dysregulation. Our case was managed successfully in the perioperative period.

We believe the dramatic presentation in our case was precipitated by acute necrosis of the tumor due to rapid growth, which in turn incited a massive inflammatory reaction mimicking acute orbital cellulitis. This case highlights a rare presentation of metastatic neuroendocrine tumor as orbital cellulitis in a young female. The ophthalmologist should be able to distinguish this from orbital infection, orbital outlet obstruction or the systemic carcinoid syndrome.

  References Top

Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer 2003;97:934-59.  Back to cited text no. 1
Pinchot SN, Holen K, Sippel RS, Chen H. Carcinoid tumors. Oncologist 2008;13:1255-69.  Back to cited text no. 2
Stroosma OB, Delaere KP. Carcinoid tumors of the testis. Br J Urol 2008;101:1101-5.  Back to cited text no. 3
Modlin IM, and Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997;79:813-29.  Back to cited text no. 4
Goldberg RA, Rootman J, Cline RA. Tumors metastatic to the orbit: A changing picture. Surv Ophthalmol 1990;35:1-24.  Back to cited text no. 5
Riddle JP, Font RL, Zimmerman LE. Carcinoid tumors of the eye and orbit: A clinicopathologic study of 15 cases, with histochemical and electron microscopic observations. Hum Pathol 1982;13:459-69.  Back to cited text no. 6
Jensen RT, Norton JA. Carcinoid tumours and the carcinoid syndrome. In: DeVita VT, Hellman S, Rosenberg SA, editors. Cancer Principles and Practice of Oncology, 5 th ed. Philadelphia: Lippincott-Raven Publishers; 1997. p. 1704-23.  Back to cited text no. 7
Harbour JW, De Potter P, Shields CL, Shields JA. Uveal metastasis from carcinoid tumor: Clinical observations in nine cases. Ophthalmology1994;101:1084-90.  Back to cited text no. 8
Kiratli H, Yilmaz PT, Yildiz ZI. Metastatic atypical carcinoid tumor of the inferior rectus muscle. Ophthal Plast Reconstr Surg 2008;24:482-4.  Back to cited text no. 9
Mehta JS, Abou-Rayyah Y, Rose GE. Orbital carcinoid metastases. Ophthalmology 2006;113:466-72.  Back to cited text no. 10
Turaka K, Mashayekhi A, Shields CL, Lally SE, Kligman B, Shields JA. A case series of neuroendocrine (carcinoid) tumor metastasis to the orbit. Oman J Ophthalmol 2011;4:125-8.  Back to cited text no. 11
[PUBMED]  Medknow Journal  
Skibell BC, Patrinely JR, Font RL. Metastatic carcinoid presenting as orbital cellulitis. Ophthalmic Pract 1998;16:88-90.  Back to cited text no. 12
Fan JT, Buettner H, Bartley GB, Bolling JP. Clinical features and treatment of seven patients with carcinoid tumor metastatic to the eye and orbit. Am J Ophthalmol 1995;119:211-8.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3]

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