Surgical Removal of Intraocular Tumors: Dismissing Old Wives’ Tales CAROL L. SHIELDS AND JERRY A. SHIELDS
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LD WIVES’ TALES ARE DUSTY ANECDOTES, OFTEN
based on superstition or exaggerated incidents, passed down from old wives to young listeners. Most believe these tales to be false and realize that they have been used to discourage behaviors, particularly in children. There is a long list of tattered old wives’ tales. Both you and we have conformed to a few of these tales such as ‘‘it’s bad luck to open an umbrella inside the house’’ and ‘‘if you crack a mirror you will have 7 years of bad luck.’’ We have wished for ‘‘the coin to fall head side up for good luck’’ and ‘‘eaten an apple a day to keep the doctor away.’’ We have avoided ‘‘sidewalk cracks to break a mother’s back’’ and never allowed ‘‘the flag to touch the ground for it is bad luck.’’ Old wives’ tales, despite their ridiculous phrasing, have influenced all of us. Old wives’ tales have crept into ophthalmology, too. ‘‘If you voluntarily cross your eyes, they will stay that way’’ obviously is untrue, but there is some truth to this. Involuntarily eye crossing needs medical attention. ‘‘Removal of the vitreous is dangerous’’ also is fiction, because vitrectomy clearly has brought vision back to millions of patients worldwide. And ‘‘never inject any medication into the eye’’ has been disproved unequivocally, because intravitreal injections have dominated vision recovery for elderly, diabetic, and infected eyes. Time and experience have corrected these fictional concepts. There are old wives’ tales in ophthalmic oncology, including ‘‘surgical removal of a melanoma will seed the tumor for future recurrence, complication, and enucleation’’ and ‘‘will leave the patient blind.’’ These have been disproved by scientific studies from Europe, the United States, and now Asia.1–7 More than 50 years ago, Stallard described his technique of ciliary body tumor removal through a scleral flap.8 Later, Peyman and Raichand and Peyman and associates performed full-thickness eye wall resection for choroidal neoplasms with useful postsurgical visual acuity in 68% of cases.3,9 Damato, Damata and associates, Char and associates, Shields and Shields, and Shields and associates have refined the technique further and with favorable outcomes, despite the difficulty of this procedure.2,4–6,10–12 See accompanying article on page 36. Accepted for publication Jan 29, 2013. From the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Inquiries to Carol L. Shields, Ocular Oncology Service, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107; e-mail:
[email protected] 0002-9394/$36.00 http://dx.doi.org/10.1016/j.ajo.2013.01.028
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In their hands, globe salvage was achieved in 71% to 81% and visual outcome was 20/40 or better in approximately 50% to 64%.2,5,6 Damato and associates demonstrated that nasal tumor location and posterior extension of more than 1.5 mm from the optic disc were factors predictive of good visual results after resection of choroidal melanoma.10 Ramasubramanian and associates studied this procedure in the pediatric population, noting globe salvage in 65% and visual acuity of 20/40 or better in 64%.13 The main goal in melanoma management is patient survival. Foulds and associates compared patients with uveal melanoma managed with tumor resection (n ¼ 157) versus enucleation (n ¼ 241) and found equivalent survival.14 A more recent matched case-control analysis by Kivela and associates compared patients with large melanoma managed with transscleral resection (n ¼ 49) versus plaque radiotherapy (n ¼ 49) and found equivalent survival.15 In that study, quality of life was similar in the 2 groups, but visual outcome was more favorable in the resection group. In this issue of the Journal, Lee and associates further confirm that surgical resection is a safe and effective therapy for eyes with intraocular tumors.7 In their analysis of 27 patients managed with resection for uveal melanoma (70%), melanocytoma (7%), epithelial adenoma (11%), and single cases (4%) of schwannoma, leiomyoma, and medulloepithelioma, globe salvage was achieved in 74% and visual acuity of 20/40 or better was achieved in 22%. Melanoma-related metastases were detected in 45% at 5 years. The surgical removal of an intraocular tumor is an extraordinarily challenging feat.4 This task requires unparalleled surgical skills for submillimeter microscopic dissection of the eye, avoiding traps of hemorrhage, retinal or lens damage, and hidden tails of tumor. Fine cautery is applied to the choroid to allow dry removal of the tumor. The most delicate step of this technique is to peel the mass from the undersurface of the retina using gentle strokes, displacing the retina inward while drawing the tumor outward. By necessity, given the small size of the globe, tumor-free margins usually are tight and are judged clinically based on tissue features and later are based on histopathologic findings. If the tumor is malignant and margins are involved, plaque radiotherapy is applied. This heroic procedure is not performed commonly for various reasons, some including large tumor basal dimension, posterior tumor location, tumor seeding into the anterior chamber or vitreous, patient requiring anticoagulation,
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and the surgeon’s perception of the procedure as too difficult or too time consuming. Complications range from transient hyphema or vitreous hemorrhage to retinal detachment or expulsive hemorrhage. Lee and associates report hyphema (30%), vitreous hemorrhage (52%), retinal detachment (53%), and no case of expulsive hemorrhage.7 Techniques of vitrectomy and retinal tamponade often are used for globe reconstruction. Despite the difficulty of local resection, it is the treatment of choice for several benign and malignant intraocular tumors. Enlarging benign tumors, such as melanocytoma, adenoma, and leiomyoma, can destroy the globe and are not particularly radiosensitive or chemosensitive. Circumscribed malignant tumors, especially those that are located
anteriorly in the eye, may be best served with resection, allowing the patient to avoid the consequences of radiotherapy. These include iris or iridociliary melanoma, adenocarcinoma of the pigmented and nonpigmented epithelium, and selected metastatic foci such as carcinoid tumor. Local resection of intraocular tumor is not for every patient and is not performed by every ocular oncologist. Patients should be appropriately selected and the surgeon should be appropriately skilled for this procedure. Future advancements in surgical tools and retinal repair and tamponade will allow for improved resection technique with fewer unwanted complications. Until then, like an old wife once said, we will ‘‘knock 3 times on wood for good fortune’’ for successful patient outcome.
ALL AUTHORS HAVE COMPLETED AND SUBMITTED THE ICMJE FORM FOR DISCLOSURE OF POTENTIAL CONFLICTS OF INTEREST and none were reported. Supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania. Involved in Design and conduct of study (C.L.S.); Collection (C.L.S.), management (C.L.S.), analysis (C.L.S.), and interpretation (C.L.S., J.A.S.) of data; and Preparation (C.L.S.), review (C.L.S., J.A.S.), and approval (C.L.S., J.A.S.) of manuscript.
REFERENCES 1. Foulds WS. The local excision of choroidal melanomata. Trans Ophthalmol Soc UK 1973;93(0):343–346. 2. Damato B. The role of eyewall resection in uveal melanoma management. Int Ophthalmol Clin 2006;46(1):81–93. 3. Peyman GA, Raichand M. Full-thickness eye wall resection of choroidal neoplasms. Ophthalmology 1979;86(6): 1024–1036. 4. Shields JA, Shields CL. Surgical approach to lamellar sclerouvectomy for posterior uveal melanomas. The 1986 Schoenberg Lecture. Ophthal Surg 1988;19(11):774–780. 5. Shields JA, Shields CL, Shah P, Sivalingam V. Partial lamellar sclerouvectomy for ciliary body and choroidal tumors. Ophthalmology 1991;98(6):971–983. 6. Char DH, Miller T, Crawford JB. Uveal tumour resection. Br J Ophthalmol 2001;85(10):1213–1219. 7. Lee CS, Rim H, Kwon HJ, Yi JH, Lee SC. Partial lamellar sclerouvectomy of ciliary body tumors in the Korean population. Am J Ophthalmol 2013;156(1):36–42. 8. Stallard HB. Partial cyclectomy. Br J Ophthalmol 1961;45(12): 797–802.
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9. Peyman GA, Juarez CP, Diamond JG, Raichand M. Ten years experience with eye wall resection for uveal malignant melanomas. Ophthalmology 1984;91(12):1720–1725. 10. Damato BE, Paul J, Foulds WS. Predictive factors of visual outcome after local resection of choroidal melanoma. Br J Ophthalmol 1993;77:616–623. 11. Shields JA, Shields CL. Intraocular Tumors. An Atlas and Textbook. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2008;546–548. 12. Shields JA, Shields CL. Intraocular Tumors. A Text and Atlas. Philadelphia: WB Saunders; 1992;188–191. 13. Ramasubramanian A, Shields CL, Kytasty C, Mahmood Z, Shah S, Shields JA. Resection of intraocular tumors (partial lamellar sclerouvectomy) in the pediatric age group. Ophthalmology 2012;119(12):2507–2513. 14. Foulds WS, Damato BE, Burton RL. Local resection versus enucleation in the management of choroidal melanoma. Eye (Lond) 1987;1(6):676–679. 15. Kivela T, Puusaari I, Damato B. Transcleral resection versus Iodine brachytherapy for choroidal malignant melanomas 6 millimeters or more in thickness: a matched case control study. Ophthalmology 2003;110(11):2235–2244.
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY 2013
Biosketch Dr Carol Shields was trained in ophthalmology at Wills Eye Hospital in Philadelphia and completed fellowship training in ocular oncology and ophthalmic pathology. She is currently Co-Director of the Oncology Service, Wills Eye Hospital, and Professor of Ophthalmology at Thomas Jefferson University in Philadelphia. Each year the Oncology Service manages approximately 500 patients with uveal melanoma, 120 patients with retinoblastoma, and numerous other intraocular, orbital, and adnexal tumors from the United States and abroad.
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