University of Iowa Health Care

Ophthalmology and Visual Sciences

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Iris Melanoma

47-year-old male referred in 1997 for evaluation of iris lesion OS

Avinash Tantri, M.D., Wallace L. M. Alward, M.D. and Thomas A. Weingeist, M.D., Ph.D.

February 21, 2005

Chief Complaint: 47-year-old male referred in 1997 for evaluation of iris lesion OS.

History of Present Illness: Lesion OS was first noticed in 1972. The patient noted increased pigmentation. He was evaluated in 1997 at the University of Iowa, and it was decided to watch this lesion. There was slow, progressive growth of a pigmented, iris lesion OS in 2004. There are no other ocular complaints or visual problems.

PMH/FH/POH: Non-contributory.

EXAM

  • Best corrected visual acuities: 20/20 OD and OS
  • Pupils: Normal OD, Corectopia OS, no RAPD
  • CVF: Full OU
  • IOP: 17 mmHg OD and 15 mmHg OS
  • EOM: Full OU
  • SLE: See photos below
  • Fundus: Normal OU
  • Echography notable for an iris mass with a maximum thickness of 1.07 mm without ciliary body involvement.
Figure 1: SLE Examination OS. Darkly pigmented lesion extending from the pupil margin to the angle causing corectopia. There is irregularity, vascularity, and elevation of the mass. There is ectropion uvea. This is an earlier photo of the patient from our photo archive. Compare this lesion with the one in Figure 2.
Iris Melanoma

Figure 2: Gonioscopy of Iris Melanoma. A) Slit lamp view of iris melanoma. There is now change in the shape, pattern, and growth of the iris lesion (compare to Figure 1). B) Gonioscopy of iris melanoma denoting mass extension to the peripheral iris, but without invasion of the trabecular meshwork.
gonioscopy of iris melanoma

Figure 3: Sentinel vessel. Slit lamp photograph of engorged vessel feeding the intraocular melanoma (sentinel vessel).
Sentinel vessel

Figure 4: An iridocyclectomy was perfomed to remove the iris lesion.

Video showing the removal of the iris lesion.


If video fails to load, download Video in Windows Media Format
Surgery performed by Wallace Alward, MD; Assisted by Thomas Weingeist, MD, PhD

Figure 5: SLE Photos OS after iridocyclectomy
Iris Defect Iris Defect

Figure 6: Hypotonous Maculopathy. A) Following the iridocyclectomy, the patient had post-operative hypotony resulting in choroidal effusions and optic nerve swelling. Hypotonous maculopathy was associated with a temporary post-operative drop in visual acuity. B) The intraocular pressure increased over a few weeks and the optic nerve swelling and hypotonous maculopathy resolved within 2-3 weeks. The visual acuity was 20/20 at one month following surgery.
Hypotonous Maculopathy

Figure 7: Pathology of Iris Melanoma
Pathology Low power view of excised iris tissue containing melanoma.
Pathology of Iris Melanoma 100X view of tumor containing both melanoma and nevus cells. Cells are invasive, pigmented, and form nests.
Pathology of Iris Melanoma 200X view of tumor, processed with bleach to remove melanin. Note large, vacuolated nuclei. Cellular structure shows atypia.
Pathology of Iris Melanoma Slide showing that the tumor does not invade through sclera.

Discussion

Iris Melanoma

The iris is an uncommon primary location for melanoma. Within uveal tissue, the most common locations for melanoma are (in descending order): choroid, ciliary body, and iris. Iris melanomas comprise 3-12% of uveal melanomas. The annual incidence is approximately 1/million.

Risk factors include Caucasian race and light iris color. Questionable risk factors include neurofibromatosis type-1 (NF-1) and sunlight exposure. The great majority of iris melanomas occur on the inferior half of the iris, which further implicates sun exposure.

Patients present in a variety of ways. Usually, the patient or ophthalmologist notes a spot on the iris which grows over time. Less common presenting signs and symptoms include increased IOP, decreased vision, and hyphema.

Iris melanomas display a variety of growth patterns. Two main categories include nodular or diffuse growth patterns. "Ring melanoma" is a term used to describe a tumor which grows around the iris in a circular shape. These tumors usually display the diffuse growth pattern. Iris melanomas can be divided into 3 histologic types: Spindle B, Epithelioid, and Mixed. Spindle B is the most common type.

Iris melanomas may present with elevated IOP, although the majority do not. Causes of increased IOP are controversial, but include the following:

  • Angle invasion by tumor
  • Pigment dispersion
  • Anatomic angle closure by tumor (rare)
  • Neovascularization following plaque radiotherapy

Elevated IOP can be treated by topical or oral medications. Treatment of the iris melanoma may help reduce IOP as well. Performing argon laser trabeculoplasty (ALT) is controversial, since it could cause dispersion of tumor cells. Some authors have suggested performing ALT well away from the tumor. Cyclodestructive procedures are also controversial. Filtration surgeries are contraindicated, because they have been associated with metastasis.

Differentiating benign iris nevi from malignant iris melanomas can be difficult. Multiple studies have sought to clarify risk factors for malignancy, which include:

  • Photographic documentation of growth
  • Size (>3mm diam X >1mm thick)
  • Glaucoma and/ or pigment dispersion
  • Invasion
  • Prominent vascularity
  • Hyphema
  • Ring growth

However, although the above risk factors are suggestive of malignancy, many of the features may be seen in some iris nevi. The following may be seen in iris nevi, and have led to enucleation in some cases:

  • Growth
  • Glaucoma or pigment dispersion
  • Ectropion Uvea
  • Hyphema
  • Diffuse or Nodular growth
  • Trans-scleral involvement
  • Angle involvement
  • Tapioca or Ring appearance
  • Vascularity
  • Cataract

Metastasis is unusual in iris melanoma. The overall rate of spread at 10 years is 3-5%. Epithelioid tumors show a slightly higher rate (7%), while mixed tumors show the highest rate (11%). This is in contrast to choroidal melanomas, which metastasize most readily if epithelioid.

The true metastatic rate for iris melanoma is probably lower. Studies documenting metastatic rate include only patients with biopsy-confirmed tumors. Many patients may have slow growing iris melanomas that are mistaken for nevi, or not recognized at all. Other patients have slow growing lesions which are thought to be iris melanomas, but are followed long term and never undergo biopsy. Therefore, it is likely that the above studies select for the most aggressive tumors.

Metastatic rate for iris melanoma is lower than ciliary body melanoma, and much lower than choroidal melanoma. When metastases do occur, the liver and lungs are the most common sites.

Studies have sought to identify risk factors for metastasis:

  • Mixed or epithelioid tumor
  • Invasion (angle or trans-scleral)
  • Increased IOP
  • Glaucoma surgeries

Diagnosis can be difficult due to aggressive features which can occasionally be demonstrated by benign nevi. The following are diagnostic options:

  • Follow lesion over time with photos (best)
  • Ultrasound biomicroscopy
  • Fine needle aspiration (FNA) – safe, but reports of false negatives
  • Excisional biopsy
  • Enucleation

Enucleation could be considered in eyes with poor vision. One example is an eye with intractable IOP rise. FNA is not associated with increased risk of metastasis. However, in a tumor composed of both nevus and melanoma cells, the FNA may pick up only nevus cells. Furthermore, in a small, slow-growing tumor distant from the angle, FNA may not change management. Excisional biopsy may be considered for smaller lesions.

It is important to consider that an apparent iris melanoma may simply be an extension of a choroidal or ciliary body melanoma. Ultrasound biomicroscopy can be particularly useful to investigate this.

Other diagnostic modalities listed below are used less often.

  • Infrared transillumination
  • Fluorescein angiography

Treatment options for iris melanomas include:

  • Observation
  • Excision
  • Enucleation
  • Plaque radiotherapy

Plaque radiotherapy is the newest treatment for iris melanoma. Because of the anterior location of iris melanoma, radiation retinopathy is not common after treatment. Cataract and iritis are the most common adverse reactions of radiotherapy.

Our patient decided to have an iridocyclectomy (see video) to remove the melanoma completely. The patient should be examined for recurrence and undergo yearly monitoring of liver function tests and chest X-rays.

Diagnosis: Iris Melanoma

EPIDEMIOLOGY

  • Yearly Incidence 1/ 1,000,000
  • 3-12% of uveal melanomas
  • Risk factors: caucasian; light iris color; NF-1; sunlight
  • Average age of onset: 40-50 years old
  • Location: Inferior half of iris

Risks factors for metastasis

  • Mixed or epithelioid tumor
  • Invasion (angle or trans-scleral)
  • Increased IOP
  • Glaucoma surgeries
  • Overall: 3-5%
  • Epithelioid: 7%
  • Mixed: 11%

SIGNS OF MALIGNANCY

  • Photo documentation of growth
  • Size (>3mm diam X >1mm thick)
  • Glaucoma and/ or pigment dispersion
  • Invasion
  • Prominent vascularity
  • Hyphema
  • Tapioca or Ring growth

SYMPTOMS

  • Spot on eye grows
  • Increased IOP
  • Decreased vision
  • Hyphema

TREATMENT

  • Follow lesion over time with photos (best)
  • FNA – safe, but reports of false negatives
  • Excisional biopsy
  • Enucleation
  • Observation
  • Excision
  • Enucleation
  • Plaque radiotherapy

Differential Diagnoses for Iris Masses/Pigmentation

  • Iris nevus
  • Melanoma
  • Choroidal or ciliary body melanoma
  • Iris cyst
  • Hemangioma
  • Metastatic CA
  • ICE syndromes
  • Iris FB
last updated: 02-21-2005
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