Ring-Shaped Limbal Tumor

Spaulding, Abbot G.; Green, W. R.; Font, Ramon L.

doi:10.1001/archopht.1967.00980020078016

Cited by 13 publications

(1 citation statement)

References 3 publications

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“…No tumour mass was identified macroscopically in the enucleated specimen and the diagnosis was only established by microscopy. This case has similarities to the diffuse malignant melanomas reported by Brown et al 18 and Spaulding et al 19. Ring tumours with unusual histology, including T cell lymphoma20 and borderline spindle cell naevus,21 have also been described.…”

Section: Discussionsupporting

confidence: 80%

Ring melanoma---a rare cause of refractory glaucoma

Lee

Cree

Hungerford

1999

British Journal of Ophthalmology

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Background-Ring melanoma of the ciliary body and iris is extremely rare and often has adverse histology. This tumour may cause raised intraocular pressure. Methods-A review of four cases of ring melanomas with insidious presentations seen in the ocular oncology service over a 12 month period. Results-All four patients presented with unilateral anterior segment abnormalities and refractory glaucoma. The misdiagnoses of the causes of the glaucoma included angle recession from previous blunt trauma (patient 1); iridocorneal endothelial (ICE) syndrome supported by endothelial specular microscopy (patients 2 and 3); and melanocytoma on ciliary body biopsy (patient 4). Two patients were treated by several cyclodiode ciliary body ablation treatments and the other two underwent trabeculectomies and Molteno tubes. Two of the four patients have since died from their disease. Conclusion-The ophthalmologist should re-evaluate the diagnosis in patients with anterior segment abnormalities and refractory ipsilateral glaucoma. Endothelial specular microscopy and biopsy of the suspicious lesion may give misleading reassurance. The potential presence of an anterior uveal melanoma must always be considered. (Br J Ophthalmol 1999;83:194-198) Ring melanoma of the ciliary body and iris was first described by the Russian ophthalmologist Ewetsky in 1898 1 who called it a ring sarcoma. It is a rare variant of the "diVuse melanoma" which is defined as a primary uveal tumour that involves more than a quarter of the uveal tract.2 In practice, ring melanomas seldom infiltrate the uveal tract as extensively, but the ciliary body is the main site of involvement. 3The ring may arise from coalescence of tumours arising from multiple sites. Materials and methodsWe reviewed four cases of ring melanomas causing unilateral refractory glaucoma which were seen in the ocular oncology clinic over a 12 month period. Results CASE 1A 23 year old white man was admitted via the accident and emergency in department of a district general hospital with a history of vomiting and malaise. A computed tomograph (CT) scan of the abdomen was performed and showed multiple liver lesions which were biopsied, and which showed poorly diVerentiated round cell tumour of uncertain origin.He was commenced on chemotherapy for extraosseus Ewing's sarcoma. At admission the right pupil was noted to be distorted and a pigmented cystic lesion was noted on the conjunctiva of the right eye. An ophthalmological opinion was sought.Further questioning revealed that the patient had sustained blunt trauma to the right eye from a tennis ball at the age of 10, and 2 years before admission had been diagnosed with glaucoma secondary to angle recession at another eye unit. Iris heterochromia had also been noted. The glaucoma was refractory to conservative treatment and he had undergone two episodes of cyclodiode ciliary body ablation 6 months earlier. The vision had deteriorated over the past year and the eye had become painful.On examination visual acuity was no perception of lig...

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Section: Discussionsupporting

confidence: 80%