|Year : 1995 | Volume
| Issue : 3 | Page : 137-140
Adenoma of nonpigmented epithelium of the ciliary body presenting as anterior uveitis and glaucoma: A case report
Jyotirmay Biswas, Arvind Neelakantan, B Sridhar Rao
Medical Research Foundation, Madras, India
Medical Research Foundation, 18 College Road, Madras 600 006
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Biswas J, Neelakantan A, Rao B S. Adenoma of nonpigmented epithelium of the ciliary body presenting as anterior uveitis and glaucoma: A case report. Indian J Ophthalmol 1995;43:137-40
|How to cite this URL:|
Biswas J, Neelakantan A, Rao B S. Adenoma of nonpigmented epithelium of the ciliary body presenting as anterior uveitis and glaucoma: A case report. Indian J Ophthalmol [serial online] 1995 [cited 2022 Jan 21];43:137-40. Available from: https://www.ijo.in/text.asp?1995/43/3/137/25289
rue tumours of the non-pigmented ciliary epithelium are extremely rare and may be congenital or acquired. Congenital tumours arise from the primitive medullary epithelium while the origin of acquired tumours is from fully differentiated ciliary epithelium. They may take the form of true neoplasms (adenomas or adenocarcinomas) or reactive proliferations (pseudoadenomatous hyperplasia). Adenomas and adenocarcinomas usually exhibit slow progressive enlargement and may present with a subluxated lens, cataract and secondary glaucoma. Some of the tumours mimic malignant melanoma or epibulbar tumour when extended extrasclerally., We report here a case of a ciliary body adenoma in a middle-aged male who presented to the uveitis clinic with features of granulomatous anterior uveitis and secondary glaucoma.
| Case report|| |
A 39-year-old male presented in July 1991 with a history of redness, watering and pain in the left eye since one and a half years. He was diagnosed as a case of anterior uveitis with secondary glaucoma and was treated with topical steroids and mydriatic. There was a history of tuberculosis in 1984, for which he received treatment for 9 months.
On examination the best-corrected visual acuity was 6/6 in the right eye and 6/36 in the left eye. Slit-lamp examination in the right eye was normal. In the left eye there was multiple mutton fat keratic precipitates, 2+ flare and 1+ cells in the anterior chamber, multiple posterior synechiae, posterior subcapsular cataract and anterior vitreous opacities. Dilated episcleral vessels (sentinel vessels) were also seen temporally. The intraocular pressure was 10 mm Hg in the right eye and 40 mm Hg in the left eye. On gonioscopy of the left eye the angle was closed except inferiorly. The right eye fundus was normal and in the left eye there was a greyish -white mass in the superotemporal quadrant behind the iris. This was confirmed by water-bath ultrasonography. The circular echogenic solid mass in the region of ciliary body measured 1.0 x 0.76 cm jutting into the vitreous cavity [Figure - 1]. There was moderate internal reflectivity within the tumour. Systemic evaluation ruled out any infection and X-ray chest was suggestive of healed tuberculosis. All haematological investigations were normal and anterior chamber tap was negative for acid-fast bacilli. Despite these negative reports, a presumptive diagnosis of tuberculoma of ciliary body was made and antitubercular therapy with oral isoniazid, ethambutol, pyrazinamide and intramuscular streptomycin was begun.
Three months later, the patient presented with severe redness, watering and pain in the left eye. The visual acuity in the left eye had decreased to perception of light with defective projection. Slit-lamp examination showed rubeosis iridis, ectropion of the uvea with flare and cells in the anterior chamber. The intraocular pressure was 32 mm Hg. A clinical diagnosis of amelanotic ciliary body melanoma was made and considering the poor visual prognosis, an enucleation of the globe was performed.
| Pathology|| |
Gross examination of a cut section of the globe revealed a large mass 6x5 mm in size arising from the ciliary body and extending into the vitreous behind the lens [Figure - 2]. There was an associated retinal detachment and deep cupping of the optic nerve.
Histopathological examination showed a tumour mass arising from the nonpigmented epithelium of the ciliary body [Figure - 3]. The tumour cells were arranged in sheets and tubules forming glandular patterns with areas of eosinophilic and basement membrane material like depositions. The cells were well differentiated without mitotic activity. The tumour cells showed intracytoplasmic Periodic Acid Schiff-(PAS) positive materials [Figure - 4]. Alcian blue and mucicarmine stain was positive suggesting acid mucopolysaccharide deposition. There was extensive iris atrophy with focal infiltration of mononuclear cells. Anterior chamber angle was closed due to peripheral anterior synechiae; red blood cells and melanin pigments in clumps were seen in the pars plana region.
| Discussion|| |
Ciliary body melanomas have been known to present sometimes with severe pain and pigment dispersion simulating uveitis with secondary glaucoma. In contrast, ciliary body adenomas are slow-growing tumours and present as incidental finding with decreased vision due to subluxation of lens, cataract or secondary glaucoma. One case of ciliary body adenoma presenting as anterior uveitis has been earlier reported by McGowan et al. This patient was a 56-year-old male who presented with a left ciliary body mass, anterior vitreous haemorrhage and a subluxated cataractous lens. There was rapid enlargement of the mass, increasing in anterior uveitis.
Fraser in a review of 450 eyes with malignant melanoma of the choroid has documented intraocular inflammation in 22 cases (4.9%). Of the 22 patients, 6 each had anterior uveitis, and panophthalmitis. The eyes with acute panophthalmitis were found to harbour necrotic choroidal melanomas. In our patient, the uveitis could be due to necrosis of the tumour inducing inflammation. Such tumours, though benign, can show active phase of growth resulting in rapid enlargement and necrosis. The other possibility is the migration of pigments from the pigmented ciliary epithelium since migration of the pigment was seen at a few places within the tumour and in the pars plana region.
The diagnosis of adenoma was proven by histology. Acid mucopolysaccharide deposition, a characteristic feature of adenomatous tumour was confirmed by special stains (PAS and alcian blue). Abundant amount of acid mucopolysaccharide production indicates an active phase of the tumour growth and was consistent with the rapid progression of the tumour in our patient.
This report along with that of McGowan et alindicate that in a situation of ciliary body mass with uveitis, an acquired tumour of the nonpigmented epithelium of the ciliary body should be kept in mind. Though the globe was enucleated in our patient, this tumour is amenable to local resection procedures such as iridocyclectomy or sclerouvectomy.,
| Acknowledgement|| |
The pathological diagnosis was confirmed by Dr. Narsing A. Rao, Director, Ophthalmic Pathology, Doheny Eye Institute, University of Southern California, Los Angeles, USA. The ultrasonography was confirmed by Dr. S. Suresh, Director, Mediscan Systems, Madras. The authors acknowledge their help.
| References|| |
Shields JA. Diagnosis and Management of Intraocular Tumors. St. Louis, CV Mosby, 1983, pp. 322-359.
Shields JA, Shields CH. Intraocular Tumors. A Text and Atlas. Philadelphia, WB Saunders, 1992, pp. 481-484.
Chang M, Shields JA, Wachrel DL. Adenoma of the nonpigmented epithelium of the ciliary body simulating a malignant melanoma. Am J Ophthalmol 88:40-44, 1979.
Jain IS, Gupta A, Ram J. Adenocarcinoma of ciliary epithelium in a young boy. Ann Ophthalmol 19:236-237, 1987.
Rodrigues M, Hidayat A, Karesh J. Pleomorphic adenocarcinoma of the ciliary epithelium stimulating an epibulbar tumor. Am J Ophthalmol 106:595-600, 1988.
Shields CL, Shields JA, Shields MB, et al. Prevalence and mechanisms of secondary intraocular pressure elevation in eyes with intraocular tumors. Ophthalmology 94:839-846, 1987.
McGowan HD, Simpson ER, Hunter WS, et al. Adenoma of the nonpigmented epithelium of the ciliary body. Can J Ophthalmol 26:328-333, 1991.
Fraser DJ Jr., Font RL. Ocular inflammation and hemorrhage as initial manifestations of uveal malignant melanoma. Incidence and prognosis. Arch Ophthalmol 97:1311-1314, 1979.
Burn PG, Maumenee AE. Iridocyclectomy for benign tumours of the ciliary body. Am J Ophthalmol 63:447-452, 1967.
Shields JA, Shields CL, Shah P, et al. Partial lamellar sclerouvectomy for ciliary body and choroidal tumours. Ophthalmology 98:971-983,1991.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]