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Year : 2004  |  Volume : 52  |  Issue : 2  |  Page : 160-2

Uveal Metastasis from a Bronchial Carcinoid Tumour

Sankara Nethralaya, Medical and Vision Research Foundation, Chennai, India

Correspondence Address:
J Biswas
Sankara Nethralaya, Medical and Vision Research Foundation, Chennai
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Source of Support: None, Conflict of Interest: None

PMID: 15283226

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A rare case of bronchial carcinoid tumour metastasis to the ciliary body and the choroid with clinical, diagnostic and histopathological correlation is reported.

Keywords: Carcinoid, uveal metastasis

How to cite this article:
Biswas J, Babu K, Krishnakumar S, Shanmugam MP. Uveal Metastasis from a Bronchial Carcinoid Tumour. Indian J Ophthalmol 2004;52:160

How to cite this URL:
Biswas J, Babu K, Krishnakumar S, Shanmugam MP. Uveal Metastasis from a Bronchial Carcinoid Tumour. Indian J Ophthalmol [serial online] 2004 [cited 2022 Sep 27];52:160. Available from: https://www.ijo.in/text.asp?2004/52/2/160/14601

Uveal metastasis is the most common form of intraocular malignancy in adults. Carcinoid tumours originate from the neuroendocrine cells of the amine precursor uptake and decarboxylation system in the gastrointestinal tract and less commonly in the bronchus, thymus, ovary, testis and thyroid. They metastasise to the lymph node (89.8%), liver (44.1%) and bone.[1] Metastasis to the eye is rare. [2],[3],[4] Even so, secondary tumours have been reported in the eye, orbit and optic nerve.[4] Uveal metastasis due to carcinoid tumours constitutes 2.2% of all uveal metastasis.[2] We report here a rare case, probably the first to be reported in the Indian literature (Medline search), of bronchial carcinoid metastasis to the uveal tract.

  Case report Top

A 33-year-old lady presented with a small growth on the iris in the right eye of one year's duration along with blurring of vision of two months' duration. There was no history of pain or redness. She had a history of middle and lower lobectomy done for a histologically proven carcinoid tumour of the intermediate bronchus 10 years earlier. She has been on a regular follow-up with her oncologist and has had no recurrence since the excision of the primary tumour. There was no history of systemic hypertension.

On examination, her best corrected visual acuity in the right eye was 6/9 and in the left eye, 6/6. Slitlamp examination of the right eye revealed a vascular pink mass arising from the ciliary body, eroding the iris root and impinging on the lens superiorly [Figure - 1]. Fundus examination of the right eye showed a discrete orange yellow choroidal lesion of about 1-2 disc diameters, inferonasal to the disc with overlying retinal detach-ment. The left eye examination was unremarkable. Intraocular pressures was 14mm Hg by applanation tonometry in both eyes.

Ultrasound examination of the right eye showed a dome-shaped mass with a smooth surface, high surface spike and moderate internal reflectivity inferonasal to the disc. Ultrasound biomicroscopy revealed a homogenous mass arising from the ciliary body between 11 and 1 o' clock. The temporal edge of the mass showed cystic changes. There was no scleral or pars plana extension.

Fine needle aspiration biopsy of the ciliary body mass showed loose clusters of tumour cells, some grouped, with oval to round nucleus and speckled nuclear chromatin suggestive of malignancy. As there was a history of bronchial carcinoid, a provisional diagnosis of metastasis to the ciliary body and choroid was made. However, it was necessary to rule out any new tumour elsewhere. A thorough systemic evaluation by an oncologist with chest Xray, computerised tomography, and ultrasound examination of the abdomen did not show the recurrence of the primary tumour, occurrence of a new tumour elsewhere or any other sites of metastasis. As histopathological confirmation was deemed necessary and after discussing various management options with the patient and the internist enucleation was done with informed consent. A cut section of the globe revealed a white mass 5mm x 7mm arising from the ciliary body and a choroidal mass of 4mm diameter [Figure - 2]. Microscopic examination showed tumour deposits in the ciliary body and the choroid. The uniform round tumour cells were arranged in a solid pattern. These cells were oval to round having prominent nuclei with finely stippled chromatin and granular cytoplasm. In some areas, the tumour cells were seen arranged around a central lumen forming a rosette-like pattern [Figure - 3]a, b and c. The groups of tumour cells were separated by small blood vessels. A diagnosis of metastatic carcinoid tumour to the ciliary body and the choroid was made on histological examination. On reviewing the histopathology slides of the primary tumour, a similarity between the primary and the metastasis was noted. At 10 months' follow-up, the patient was doing well with no recurrence or appearance of new lesions.

  Discussion Top

The term "Karzinoid" was coined by Oberndorfer in 1907. Carcinoid tumours constitute 1 to 3% of all bronchopulmonary cancers.[1] Bronchial carcinoids have a tendency to undergo metastasis to the uveal tract whereas gastrointestinal carcinoids have a predilection for the orbit. [2],[3],[4] The predominant site of uveal metastasis is the choroid.

In this case, the diagnosis of metastasis to the ciliary body and the choroid was strongly supported by prior history of bronchial carcinoid and fine needle aspiration biopsy. Secondary tumours may be the initial manifestation of a primary tumour elsewhere. [4],[5] Thus an appropriate systemic workup is mandatory.

The long interval between the primary and the appearance of secondaries indicate that carcinoids tend to be relatively indolent. Archer and Gardiner,[4] in their series, reported the longest duration of 9 years between the primary and the secondary appearances. Our patient had a latent interval of 10 years. Despite metastasis, carcinoid tumours often grow slowly and are associated with long survival, if detected early. A distinctive orange colour of metastatic choroidal carcinoid deposits have been described in the literature. This may help in the diagnosis.[2]

Histopathological examination is necessary for confirmation. The secondary tumours almost always retain the histological characteristics of the primary tumours.[4] Zimmerman et al[3] described four patterns: Pattern A (solid lobules), Pattern B (trabecular or cord like), Pattern C (tubular or rosette) and Pattern D (mixed). The tumour cells are separated by fibrovascular septae and have hyperchromatic, eccentric nuclei with finely stippled chromatin and eosinophilic granular cytoplasm. They contain neurosecretory granules in their basal cytoplasm demonstrated by argyrophilic stains. However, neurosecretory granules are commonly absent in carcinoid tumours from bronchus and upper GI tract and this may lead to negative argentaffin and argyrophilic reactions.[2]

Treatment by external beam radiotherapy, local resection, photocoagulation, proton beam irradiation, radon ring brachytherapy has been described with varied degrees of success. [2],[4] Failing such treatment, and where the diagnosis is in doubt, enucleation is regarded the best procedure.[4] The options of management in our patient included resection of the ciliary body mass along with plaque treatment or transpupillary thermotherapy of the choroidal mass. As plaque was not available and as there was sufficient concern of metastasis to other parts of the body, it was decided to enucleate the eye.

Unlike the poor prognosis in patients with metastatic uveal cancer, prognosis for survival after diagnosis of intraocular metastasis from carcinoid tumour is generally good with a five-year survival rate ranging from 57% to 95%.[4] Prognosis is enhanced if the disease is recognised early and the primary and accessible metastases are removed.

  References Top

Godwin JD II. Carcinoid tumors. An analysis of 2,837 cases. Cancer 1975;36:560-69.  Back to cited text no. 1
Harbour JW, Potter PD. Uveal metastasis from carcinoid tumor -clinical observations in nine cases. Ophthalmology 1994;101:1084-90.  Back to cited text no. 2
Zimmerman LE, Stangl R, Riddle PJ. Primary carcinoid tumor of the orbit. A clinicopathologic study with histochemical and electron microscopic studies. Arch Ophthalmol 1983;101:1395-98.  Back to cited text no. 3
Archer DB, Gardiner TA. An ultrastructural study of carcinoid tumor of the iris. Am J Ophthalmol 1982;94:357-68.  Back to cited text no. 4
Eagle RC, Ehya H, Shields JA, Shields CL. Choroidal metastasis as the initial manifestation of a pigmented neuroendocrine tumor. Arch Ophthalmol 2000;118:841-45.  Back to cited text no. 5


  [Figure - 1], [Figure - 2], [Figure - 3]


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