| Patient Education |
|
Click here for patient education.
|
|
You are in: eMedicine Specialties >
Ophthalmology > IRIS AND CILIARY BODY
Leiomyoma, Iris
Article Last Updated: Dec 27, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Manolette R Roque, MD, MBA, DPBO, FPAO, President and CEO, Chief of Service, Ocular Immunology and Uveitis, Consulting Staff, Cornea and Refractive Surgery, Eye Republic Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines Co; Consulting Staff, CME Liaison, Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center
Manolette R Roque, MD, MBA, DPBO, FPAO, is a member of the following medical societies: American Academy of Ophthalmic Executives, American Society of Cataract and Refractive Surgery, American Society of Ophthalmic Administrators, American Uveitis Society, International Ocular Inflammation Society, Philippine Medical Association, Philippine Ocular Inflammation Society, and Philippine Society of Cataract and Refractive Surgery
Coauthor(s):
Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, EYE REPUBLIC Ophthalmology Clinic;
C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institute
Editors: Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine; Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles; Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Hospital; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Author and Editor Disclosure
Synonyms and related keywords:
iris leiomyoma, leiomyoma of the iris, benign intraocular smooth muscle tumor
Background
Leiomyoma of the iris is a rare, benign intraocular smooth muscle tumor. The tumor often originates from the sphincter or less commonly from the dilator muscles. Studies have found that leiomyomas occur much less frequently than previously reported. It is believed strongly that iris leiomyoma has been previously overdiagnosed. Stricter diagnostic criteria involving electron microscopy and immunohistopathology have been added as standards for reporting.
Pathophysiology
Uncertainty to the pathogenesis of iris leiomyoma exists. Hormones are speculated to play a role in the pathogenesis due to the tumor's similarity to uterine leiomyoma (fibroids) and its predilection to females. Structures in the iris of neuroectodermal origin believed to give rise to the tumor include the sphincter muscle and the dilator muscle. Structures in the iris of mesodermal origin believed to give rise to the tumor include mesenchymal tissue.
Frequency
United States
The incidence of smooth muscle tumors has been reported as 2.3%, 4%, 9%, and 14.5%, in different published series of iris tumors.
Mortality/Morbidity
No reported data on mortality and morbidity from iris leiomyoma exist. Morbidity may be limited to the occasional presence of secondary glaucoma and decreased vision when the mass reaches the visual axis.
Race
This condition is reported only in white subjects.
Sex
Females are affected more frequently than males.
Age
Cases of leiomyoma of the iris have been seen in patients aged 10-77 years.
History
This tumor is usually a benign iris mass that remains clinically stationary for many years. See Physical.
Physical
- Leiomyoma of the iris is a localized, flat to slightly elevated mass, often at the region of the sphincter muscle. It is found less commonly in the iris periphery and the anterior chamber angle.
- Clinically, the tumor appears nonpigmented (sometimes lightly pigmented), transparent (grayish white to pink colored), and vascular.
- Ectropion iridis is a common finding in the area of the tumor.
- Leiomyoma of the iris may be difficult to differentiate from an amelanotic iris melanoma.
- These tumors can be clinically stationary for many years.
Causes
The cause of leiomyoma of the iris is unknown. See Pathophysiology.
Foreign Body, Intraocular
Juvenile Xanthogranuloma
Melanoma, Ciliary Body
Melanoma, Iris
Other Problems to be Considered
Amelanotic melanoma of the iris
Foreign body in the iris
Peripheral anterior synechiae
Lymphoid hyperplasia
Iridoschisis
Iridic neovascularization
Atypical vessels of the iris
Hemangioma
Siderosis
Hemosiderosis
Iridic abscess
Imaging Studies
- Ultrasound biomicroscopy (UBM) may be helpful for evaluating this tumor.
- Electron microscopy shows the characteristic features of a smooth muscle neoplasm (thin basement membrane; plasmalemmal vesicles; and numerous, longitudinally aligned cytoplasmic filaments with scattered associated densities).
- Fluorescein angiography
- Hyperfluorescence is noted in the peripheral part of the tumor.
- Reported as a preoperative mapping procedure in the 1970s, fluorescein angiography is perceived to be of limited value today.
Other Tests
- Immunohistochemistry
- The findings are consistent with a myogenic tumor.
- The tumor cells are positive for smooth muscle actin and desmin.
- The tumor cells are negative for S-100 and melanin.
Procedures
- Fine-needle aspiration biopsy
- This test may be performed when clinical suspicion is high.
- The sample may be sent for cytologic study with the use of electron microscopy and immunohistochemistry.
- Its value may be limited because of the small sample obtained.
- Transillumination
- A leiomyoma transmits more light than a melanoma.
- The more peripheral part of the tumor may be translucent.
- Even amelanotic melanomas contain small amounts of melanin sufficient to cast a relative shadow.
Histologic Findings
Light microscopy shows the characteristic appearance of a leiomyoma with interlacing, densely packed, elongated, spindle-shaped cells, with long oval nuclei that tend to be arranged in a palisading manner, and granular eosinophilic cytoplasm of the cells and myofibrils.
Medical Care
Regular photographic documentation of the iris mass is necessary.
Surgical Care
- Conservatism is suggested in the management of demarcated iris tumors, particularly for those tumors in the central part of the iris.
- When the visual field is threatened, complete excision of the tumor in the pupillary zone is safe.
- Pupillary repair and reconstruction may be warranted on some occasions.
- In primary ciliary body leiomyoma extending into the iris and the anterior chamber, sclerouvectomy has been found to be beneficial.
Consultations
- Ocular pathologist
- Having the opinion of a subspecialist may prove worthwhile. Appropriate photographic documentation is necessary.
- The specimen obtained during biopsy should be appropriately submitted to an ocular pathologist for proper immunohistochemistry and ultrastructural study.
Further Inpatient Care
- In some centers, admission may be necessary after performing an excisional biopsy.
- Monitoring for infection, wound leakage, anterior chamber depth, and pupillary status is required.
Further Outpatient Care
- Once a diagnosis of leiomyoma is confirmed by ultrastructural analysis after excisional biopsy, no further treatment of this benign lesion is necessary.
- If the excisional biopsy is large, diplopia and glare may potentially become complaints. Addressing these issues appropriately is necessary.
In/Out Patient Meds
- Aside from the use of an eye shield, oral painkillers, and topical antibiotics and steroids after the procedure, no further special care is necessary.
- Appropriate topical ophthalmic medications may be provided in the advent of an excisional biopsy.
Complications
- Continued tumor growth may cause secondary glaucoma.
- When the mass reaches the visual axis, a decrease in vision may occur.
Prognosis
- The prognosis for vision depends on the location and size of the tumor and is usually excellent.
- The prognosis for life is excellent because these tumors are usually benign, slow-growing lesions with no known tendency for metastasis.
Patient Education
- At least annual visits with an ophthalmologist are advised for the patient.
Medical/Legal Pitfalls
- Leiomyoma of the iris is difficult to distinguish from an amelanotic stromal melanocytic neoplasm from both a clinical and a histopathologic standpoint. For this reason, progressively enlarging iris masses must be treated as potentially malignant, regardless of their clinical appearance. The use of electron microscopy is necessary in achieving proper differentiation. (See Imaging Studies.)
- Improper diagnosis, in this day and age of electron microscopy and immunohistochemistry, may lead to potential legal disputes. It is very difficult, nearly impossible, to differentiate between spindle cell tumors such as leiomyomas, Schwann cell tumors, and some melanocytic tumors by means of light microscopy only.
Special Concerns
The author was a fellow and affiliated with the Ocular Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, while performing this work.
| Media file 1:
Leiomyoma of iris. (A) Spindle cell tumor arising in region of sphincter muscle of iris. Hematoxylin and eosin, X30. (B and C) Tumor is composed of uniform spindle-shaped cells with an abundant admixture of fibrillary cell processes. Hematoxylin and eosin, X500. Armed Forces Institute of Pathology Acc. 68490. |
 |  View Full Size Image | |
Media type: Image
|
| Media file 2:
(A) Incidental iris leiomyoma (arrow) in 16-year-old female with uveitic glaucoma removed at time of glaucoma procedure. Courtesy of Alan L. Robin, MD. (B) Tumor (between arrows) is composed of spindle-shaped cells with an abundant admixture of fibrillar material. Hematoxylin and eosin, X340. (C) The cells stain positively for smooth muscle actin. Immunoperoxidase, X340. EP 96159. |
 | View Full Size Image | |
Media type: Image
|
- Ashton N, Wybar K. Primary tumours of the iris. Ophthalmologica. 1966;151(1):97-113. [Medline].
- Biswas J, Kumar SK, Gopal L, Bhende MP. Leiomyoma of the ciliary body extending to the anterior chamber: clinicopathologic and ultrasound biomicroscopic correlation. Surv Ophthalmol. Jan-Feb 2000;44(4):336-42. [Medline].
- Brovkina AF, Chichua AG. Value of fluorescein iridography in diagnosis of tumours of the iridociliary zone. Br J Ophthalmol. Mar 1979;63(3):157-60. [Medline].
- Campbell RJ, Min KW, Bolling JP. Skeinoid fibers in mesectodermal leiomyoma of the ciliary body. Ultrastruct Pathol. Nov-Dec 1997;21(6):559-67. [Medline].
- De Buen S, Olivares ML, Charlín C. Leiomyoma of the iris. Report of a case. Br J Ophthalmol. May 1971;55(5):353-6. [Medline].
- Eide N, Farstad IN, Rřger M. A leiomyoma of the iris documented by immunohistochemistry and electron microscopy. Acta Ophthalmol Scand. Aug 1997;75(4):470-3. [Medline].
- Foss AJ, Pecorella I, Alexander RA, Hungerford JL, Garner A. Are most intraocular "leiomyomas" really melanocytic lesions?. Ophthalmology. May 1994;101(5):919-24. [Medline].
- Ishigooka H, Yamabe H, Kobashi Y, Nagata M. Clinical and pathological status of mesectodermal leiomyoma of the ciliary body. A case report and review of the literature. Graefes Arch Clin Exp Ophthalmol. 1989;227(2):101-5. [Medline].
- Jellie HG, Gonder JR, Willis NR, Green L, Tokarewicz AC. Leiomyoma of the iris. Can J Ophthalmol. Jun 1989;24(4):169-71. [Medline].
- Li ZY, Tso MO, Sugar J. Leiomyoepithelioma of iris pigment epithelium. Arch Ophthalmol. Jun 1987;105(6):819-24. [Medline].
- Lowe RF, Greer CH. Benign epithelioma of the iris. A clinico-pathological case report. Br J Ophthalmol. Nov 1971;55(11):773-6. [Medline].
- Noor Sunba MS, Rahi AH, Garner A, Alexander RA, Morgan G. Tumours of the anterior uvea. III. Oxytalan fibres in the differential diagnosis of leiomyoma and malignant melanoma of the iris. Br J Ophthalmol. Nov 1980;64(11):867-74. [Medline].
- Okamoto N, Sotani T, Shimo-Oku M, Sashikata T. A case with leiomyoma of iris extirpated with cryosurgery and its pathological findings. Acta Ophthalmol (Copenh). Apr 1982;60(2):183-9. [Medline].
- Orsoni JG, Daicker B, Cardillo Piccolino F. Mesectodermal leiomyoma of the ciliary body extending into the anterior chamber. Ophthalmologica. 1985;191(2):127-9. [Medline].
- Palm E, Linder B. Excision of tumours in the iris and the ciliary body. Acta Ophthalmol (Copenh). 1968;46(3):521-8. [Medline].
- Peyman GA, Alghadyan A, Peace JH. A contact Nd:YAG laser to resect large ciliary body and choroidal tumors. Int Ophthalmol. Oct 1987;11(1):55-61. [Medline].
- Price MJ, Bell RA, Willis WE, Whiteman DW. Tapioca melanoma of the iris: clinicopathological correlation with results of fluorescein angiography. Can J Ophthalmol. Oct 1981;16(4):195-9. [Medline].
- Sevel D, Tobias B. The value of fluorescein iridography with lleiomyoma of the iris. Am J Ophthalmol. Sep 1972;74(3):475-8. [Medline].
- Shields JA, Sanborn GE, Augsburger JJ. The differential diagnosis of malignant melanoma of the iris. A clinical study of 200 patients. Ophthalmology. Jun 1983;90(6):716-20. [Medline].
- Tuncer S, Peksayar G, Demiryont M, Gozum N. Longterm follow-up of a patient with iris leiomyoma treated with partial lamellar iridocyclectomy. Acta Ophthalmol Scand. Feb 2004;82(1):112-4. [Medline].
- Yu DY, Cohen SB, Peyman G, Tso MO. Mesectodermal leiomyoma of the ciliary body: new evidence for neural crest origin. J Pediatr Ophthalmol Strabismus. Nov-Dec 1990;27(6):317-21. [Medline].
Leiomyoma, Iris excerpt Article Last Updated: Dec 27, 2007
|
