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Anterior segment tumors typically derive from the iris and ciliary body and rarely from the cornea or lens. Iris tumors tend to be smaller and visible, so they can be detected early, and rarely metastasize. In contrast, the early diagnosis of a ciliary body tumor is often very difficult because of its concealed location. Hidden behind the iris, ciliary body tumors are usually only detected after they have become relatively large, often causing treatment to be delayed until prognoses are less than ideal.1,2
The diagnosis of ciliary body tumors involves a detailed medical history and complete ophthalmic examinations, which typically include best corrected visual acuity (BCVA), tonometry, slit-lamp biomicroscopy, gonioscopy and ophthalmoscopy. Ancillary examinations include B-ultrasound examinations, high-resolution ultrasound biomicroscopy (UBM), computerized tomography, and magnetic resonance imaging. The final diagnoses of ciliary body tumors should be confirmed by pathologic examination after tumors are excised.3
There are various treatment methods available to those physicians who care for patients with ciliary tumors. In practice, these treatments are based on the type of tumor and the condition of the eye. In general, treatment of ciliary tumors has trended toward ocular conservation, including observation, local resection, and radiation therapy.4 Enucleation is typically reserved for large tumors or eyes with untreatable glaucoma.5,6 As new modalities of therapy offer lower morbidity when compared with enucleation, more physicians and patients have opted for eye and vision-sharing treatments.7
The main method of local resection of ciliary body tumors is lamellar sclerouvectomy. In this procedure, the tumor base is delineated and a free margin of 2–3 mm is outlined. A scleral flap is prepared over the outlined area. The deep scleral lamella is incised down to the choroid and the tumor is lifted with the sclera and dissected from the retina. The main indications for lamellar sclerouvectomy are ciliary body tumors and iridociliary tumors. The main contraindications are involvement of more than one-third of the ciliary body or anterior chamber angle, significant extraocular extension, and contraindication for hypotensive anesthesia. The main complications observed involve vitreous and choroidal hemorrhage, retinal detachment, cataracts and residual tumors.8
The other method of local resection of ciliary body tumors is full-thickness eye-wall resection. It is performed similarly to lamellar sclerouvectomy but the tumor is resected en bloc. The excised tissue includes all layers of the eye wall beneath the tumor, and includes the overlying retina. A tectonic scleral or corneoscleral graft is used to repair the defect. A vitrectomy is carried out at the end of the surgery to remove any vitreous incarceration or blood from the excision site. The complications are similar to those observed for lamellar sclerouvectomy.9
From January 1996 to June 2001, 22 patients (22 eyes) with ciliary tumors underwent local excision in our hospital, and were followed for 5 to 11 years. The clinical features, pathological types and follow-up outcomes were analyzed with a prospective method.
METHODS
Patients
This study had a prospective cohort design. Patients whose tumors were greater than 16 mm at their largest base diameter or metastatic were excluded from this study. A total of 22 patients (22 eyes) with ciliary body tumors conforming to the criteria were enrolled in this study from January 1996 to June 2001. The average age of the patients at the time of surgery was 36.2 years old (range, 15–52 years), with 14 women and 8 men. The right eye was involved in 11 cases, the left eye in 11 cases. All patients received local tumor excision surgery by the same ophthalmologist and were followed up every three months after the surgery for 5–11 years.
Eye examinations
All the patients received complete ophthalmic examinations before surgery, including BCVA, tonometry, slit-lamp biomicroscopy, gonioscopy, and ophthalmoscopy. Ancillary examinations included UBM, B-ultrasound and magnetic resonance imaging.
Surgical procedure
All the operations were completed by the same ophthalmologist. General hypotension anesthesia was adopted during the surgery. In each case, the surgeon performed a 360-degree peritomy at the limbus, exposing the sclera around the tumor was exposed. A transillumination test was used to define the tumor margin. A scleral slab was made from the edge of the cornea to approximately 2–3 mm from the margin of the tumor. A full thickness sclera slab was made 1 mm away from the tumor. The incised sclera, iris and the ciliary body tumor were severed from the eyeball; the sclera slab was then covered immediately and sutured discontinuously. Sclera grafts obtained from healthy donors were used to strengthen the operated regions. In patients with severe vitreous hemorrhage and vitreous leakage, closed vitrectomies were performed, and filled with expansion gas or silicon oil.10 In two patients, a local ciliary body slab excision was performed; further vitrectomy with expansion gas filling was performed in 13 patients and silicon oil filling was performed in 7 patients.
Histopathological examination
The tumors were fixed in 10% neutral buffered formalin. Sections were cut into 5 µm thicknesses and stained with hematoxylin and eosin. The slides were observed and photographed under light microscopy.
Follow-up method
The patients were followed up every two weeks in the first month after surgery, once a month for the next 6 months, and every 3–6 months thereafter. The routine eye examinations included BCVA, tonometry, slit-lamp biomicroscopy, gonioscopy, fundus examinations, B-ultrasound and UBM examinations. Chest X-ray and abdomen ultrasound were also performed to exclude tumor migration and extension.11
Ethics statement
We certified that all applicable institutional and governmental regulations concerning the ethical use of human patients were followed during this research.
RESULTS
Before surgery, the visual acuity ranged from 0.05 to 0.80 (logarithm of the minimum angle of resolution), including four patients (18.18%) with visual acuity less than 0.05, five patients (22.73%) between 0.05 and 0.30 and thirteen patients (59.09%) better than 0.30. Slight protrusion of the peripheral iris and narrowed anterior chamber angles could be seen in most patients. Through a dilated pupil, the hemispherical, brownish-black tumor behind the iris or the lens could be found. Local opacity or indention of the lens occurred in six cases; total opacity of the lens occurred in three cases; exudative retinal detachment occurred in four cases; and iris distortion occurred in four cases. One patient had scleral change and two patients had ocular hypertension, measured at 28 mmHg and 37 mmHg respectively.
UBM showed there were local semisphere eminentia with sharp borders in the ciliary body. The mean base diameter of the tumors was 12.02 mm (range: 4–16 mm).
The average follow-up time after surgery was 9.7 years (range 5–11 years). The final BCVA varied from 0.02–1.00, including 18 patients (81.82%) who had BCVA equal to or better than before surgery, 7 patients (31.82%) who had BCVA from 0.05–0.30, and 15 patients (68.18%) who had final BCVA better than 0.30. No patient had eyeball atrophy. Six patients retained circular pupils. Two patients had IOP lower than 10 mmHg. Anterior chamber artificial lenses were implanted in 3 patients. Four patients received silicon oil extractions half a year after the first surgery. Twenty-one patients (95.45%) had well-attached retinas; only one patient had a local retinal detachment. No tumor recurrence was found in 21 patients (95.45%) by slit lamp examinations, UBM and B ultrasound scanning. One patient with a melanocytoma tumor had a recurrence seven years after surgery; enucleation of the eyeball was then performed. One patient with malignant ciliary body melanoma, who had the largest tumor in the study group (16 mm in diameter) suffered from hepatic metastasis and died five years after surgery. Pathological examination showed that malignant ciliary body melanomas were found in 9 patients (40.91%) (Figure 1). Four patients (18.18%) had melanocytoma (Figure 2), 6 patients (27.27%) nonpigmented ciliary epithelial adenoma (Figure 3), 2 patients (9.09%) neurofibroma (Figure 4), and 1 patient (4.55%) neurinoma.
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Figure 1. Malignant melanoma of the ciliary body. A: Slit lamp shows a tumor of brownish-black color from the dilated pupil with cloudy lens; B: UBM shows the spherical tumor of the ciliary before surgery; C: Slit lamp shows no tumor from the dilated pupil after surgery; D: UBM image shows no tumor recurrence of the ciliary after local excision surgery; E: The whole tumor with the lamellar sclera after local excision; F: HE staining confirms the diagnosis of malignant melanoma (original magnification ×200). |
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Figure 2. Melanocytoma of the ciliary body. A: Slit lamp shows a tumor of yellow color from the dilated pupil; B and C: B ultrasound show the tumor of the ciliary body before surgery; D: UBM image of the ciliary body tumor before surgery; E: Slit lamp shows no tumor from the dilated pupil after local excision and vitrectomy surgery; F: The retina was well attached after surgery with little blood; G: The whole tumor with the lamellar sclera after local excision; H: HE staining confirms the diagnosis of melanocytoma tumor of the ciliary (original magnification×200). |
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Figure 3. Nonpigmented ciliary epithelial adenoma. A: Slit lamp shows the narrowed inferior chamber angle and the cloudy lens; B: Slit lamp shows no tumor from the dilated pupil after local excision and vitrectomy surgery; C: The eye fundus is stable with little remaining silicone oil after surgery; D: HE staining confirms the diagnosis of nonpigmented ciliary epithelial adenoma (original magnification×200). |
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Figure 4. Neurofibroma of the ciliary. A and B: Slit lamp shows the tumor in the inferior part of the eye with severe opacity of the lens; C: UBM showed the ciliary body tumor and the closed chamber angle before surgery; D: Slit lamp shows no tumor from the dilated pupil after local excision surgery; E: UBM image shows no tumor recurrence of the ciliary body after local excision surgery; F: HE staining confirms the diagnosis of neurofibroma of the ciliary body (original magnification×200). |
DISCUSSION
Ciliary tumors are very difficult to diagnose in early phases because of their special anatomical position. Tumor location, histological origin, pathological type and well-considered differential diagnoses are very important for determining the treatment, surgical protocol and prognosis.12 The life expectancy and general health of the patient should also be considered. It has been thought that most ciliary tumors are malignant melanomas and melanocytoma tumors, which are always brownish-black or chocolate brown in color. Treatments of ciliary tumors include local resection and ophthalmic radiation therapy. Enucleation of the eyeball is typically reserved for blind eyes, eyes experiencing serious intraocular complications or severe hemorrhage, and eyes containing extremely large tumors with associated exudative retinal detachment.
In the 22 patients in this study, tumor locations were determined by the UBM. UBM is a non-invasive examination for diagnosing ciliary body tumors. It is essential for early diagnosis of ciliary body tumors and the selection of therapeutic options.13-15 It can demonstrate clearly the anterior chamber angle, posterior chamber angle and the ciliary body structure. Compared with the color doppler imaging, it can also show whether ciliary body tumors have damaged the iris, and is the best choice for accurately measuring tumor size and position before surgery, and for recurrence monitoring after surgery.16
Stallard17 was the first to use the local excision surgery to treat uveal melanoma in the 1960s. With advances in vitrectomy techniques, local excision of the ciliary body can be used to treat ciliary body tumors in patients without vitreous implantation, with tumors that cover less than 4–5 hours, with tumor diameters less than 15 mm, in an otherwise healthy patient with no ocular or systematic migration who retains at least some vision.18
Our results showed that local ciliary body excision is a safe method for treating ciliary tumors. Not only can it save the eyeball, but more importantly, it could save vision. Out of the 22 patients in our study, 7 patients achieved visual acuity between 0.05–0.30 after the surgery, and 15 patients had visual acuity better than 0.30. Melanocytoma tumor recurred in only one case at seven years after surgery; enucleation of the eyeball was then performed. One patient, who had a malignant melanoma with a diameter greater than 15 mm removed from the ciliary body, suffered from hepatic metastasis and died five years after surgery. These results imply that, for several types of ciliary tumors including malignant melanoma, local excision is a suitable method.
Patients with local tumor excision surgery often experience loss of vision due to intraocular hemorrhage, vitreous prolapse and retinal detachment. It is difficult to avoid intraocular hemorrhage or vitreous prolapse, but it is relatively easy to locate the tumor accurately with vitrectomy and to excise the tumor completely with reduced complications and improved surgical success rate. Ocular shape can be maintained by replacing excised tissue with sclera grafts. Vitreous hemorrhage is a serious complication. By electrocoagulation, perfluorocarbon, and general hypopiesia perfusion anesthesia during surgery, hemorrhage rate can be decreased. Extensive excision of the ciliary body can cause postoperative low intraocular pressure. Two patients in this study with tumor bodies larger than 4 hours had postoperative IOP of less than 10 mmHg. Therefore, it might be much safer to excise only ciliary body tumors that occupy less than 4 hours. It was reported than for the choroidal melanoma of median size (i.e., the largest base diameter less than 16 mm and the height between 2.5 mm and 10 mm), a single treatment of iodine-125 (125I) brachytherapy had an 18% cumulative mortality, and a 12.5% recurrence rate after 5-year follow-up.19 Though it was reported that adjunctive plaque radiotherapy could lower the tumor recurrence rate after local resection, we didn't use this method, because the placement of a radioactive plaque at the end of local resection may be difficult if the scleral flap is very large, or if external plombage had been applied, and may be undesirable if the eye has been filled with gas. Furthermore, radiotherapy may cause optic neuropathy, maculopathy, or cataracts. There are still other ways of preventing tumor recurrence such as excising the tumor with wider clearance margins, with the aid of larger scleral openings, greater ocular decompression, adjunctive laser photocoagulation, and cryotherapy.20
For several reasons, we did not routinely elect to observe darkly pigmented lesions of the ciliary body in this study. First, it may be hard to clinically distinguish a melanocytoma of the ciliary body from a malignant melanoma. Furthermore, the characteristic angle involvement and the frequent occurrence of necrosis favor iridocyclectomy as the treatment of choice. If growth were to occur during the observation period, the enlarged lesion would necessitate a larger eye wall resection. With these considerations in mind, we think local iridocyclectomy is the preferred choice for management. If the tumor is localized and does not extend through the sclera, the iridocyclectomy is performed with a lamellar corneoscleral resection. For tumors involving the entire scleral wall, a full thickness corneoscleral resection and iridocyclectomy should be performed with a full-thickness corneoscleral graft.
In summary, UBM is an important and useful instrument for the diagnosis and treatment of ciliary body tumors. Long-term follow-up shows local excision is an effective and safe method to save the vision of such patients, especially in the early stages.
REFERENCES
1. Marigo FA, Finger PT. Anterior segment tumors:current concepts and innovations. Surv Ophthalmol 2003; 48: 569-593.
2. Lorusso FJ, Boniuk M, Font R. Melenocytoma (magnocellular nevus) of the ciliary body: report of 10 cases and review of the literature. Ophthalmology 2000; 107: 795-800.
3. Ayres B, Brasil OM, Klejnberg C, Moura LR, Fernandes BF, Burnier MN Jr. Ciliary body medulloepithelioma: clinical, ultrasound biomicroscopic and histopathologic correlation. Clin Exp Ophthalmol 2006; 34: 695-698.
4. Damato B. Developments in the management of uveal melanoma. Clin Experiment Ophthalmol 2004; 32: 639-647.
5. Biswas J, D'Souza C, Shanmugam MP. 7 diffuse melanotic lesion of the iris as a presenting feature of ciliary body melanocytoma: report of a case and review of the literature. Surv Ophthalmol 1998; 42: 378-382.
6. Isager P, Ehlers N, Overgaard J. Prognostic factors for survival after enucleation for choroidal and ciliary body melanomas. Acta Ophthalmol Scand 2004; 82: 517-525.
7. Collaborative Ocular Melanoma Study Group. The Collaborative Ocular Melanoma Study (COMS) randomized trial of iodine 125 bradytherapy for chroidal melanoma, III: initial mortality findings. COMS report No. 18. Arch Ophthalmol 2001; 119: 969-982.
8. Rummelt V, Naumann GO, Folberg R, Weingeist TA. Surgical management of melenocytoma of the ciliary body with extrascleral extension. Am J Ophthalmol 1994; 117: 169-176.
9. Simpson ER. Ciliary body melanoma: a special challenge. Can J Ophthalmol 2004; 39: 365-371.
10. Shanmuganathan VA, Newman DK. Delayed recurrence of ciliary body melanoma following local resection. Eye 2004; 18: 755-756.
11. Kujala E, Makitie T, Kivela T. Very long-term prognosis of patients with malignant uveal melanoma. Invest Ophthalmol Vis Sci 2003; 44: 4651-4659.
12. McLean IW, Saraiva VS, Burnier MN Jr. Pathological and prognostic features of uveal melanomas. Can J Ophthalmol 2004; 39: 343-350.
13. Kawana K, Okamoto F, Nose H, Oshika T. Ultrasound biomicroscopic findings of ciliary body malignant melanoma. Jpn J Ophthalmol 2004; 48: 412-414.
14. Mohamed MD, Gupta M, Parsons A, Rennie IG. Ultrasound biomicroscopy in the management of melanocytoma of the ciliary body with extrascleral extension. Br J Ophthalmol 2005; 89: 14-16.
15. Pavlin CJ, McWhae JA, McGowan HD, Foster FS. Ultrasound biomicroscogy of anterior segment tumors. Ophthalmology 1992; 99: 1220-1228.
16. Yang W, Hu S, Wang J, Wang L, Zheng B. Color doppler imaging diagnosis of intra-ocular tumor. Chin Med J 1997; 110: 664-666.
17. Stallard HB. Partial iridocyclectomy and sclerectomy. Br J Ophthalmol 1966; 50: 656-659.
18. Shield JA, Shield CJ, Shan P, Sivalingam V. Partial lamellar sclerouvectomy for cilliary body and choroidal tumors. Ophthalmology 1991; 98: 971-983.
19. Jampol LM, Moy CS, Murray TG, Reynolds SM, Albert DM, Schachat AP, et al. The COMS randomized trial of iodine 125 brachytherapy for choroidal melanoma: IV. Local treatment failure and enucleation in the first 5 years after brachytherapy. COMS report No. 19. Ophthalmology 2002; 109: 2197-2206.
20. Damato BE, Paul J, Foulds WS. Risk factors for residual and recurrent uveal melanoma after trans-scleral local resection. Br J Ophthalmol 1996; 80: 102-108.
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