Case #10: Anterior Uveal Melanoma with Extrascleral Extension

By Paul T. Finger, MD

History

This tumor exhibits 3 signs consistent with ciliary body melanoma within the eye causing displacement of the iris root, a sentinel vessel and extrascleral extension.
This tumor exhibits 3 signs consistent with ciliary body melanoma within the eye causing displacement of the iris root, a sentinel vessel and extrascleral extension.

This ciliary body melanoma can cause displacement of the iris root, a sentinel vessel and extrascleral extension. This 38 year-old patient was referred with a large “eye melanoma” with a small plaque of extrascleral extension.

Impression

Anterior Uveal Melanoma with Extrascleral Extension.
10 and 20 MHz Ultrasonography. High frequency ultrasound (UBM) reveals displacement of the iris root (arrow). Though no sclerostomy is seen, the inner and outer scleral borders are poorly defined. 10 MHz ultrasonography shows an irregularly shaped tumor > 16 mm in diameter.

*Note*

10 and 20 MHz Ultrasonography. High frequency ultrasound (20 MHz) reveals displacement of the iris root (arrow). Though no sclerostomy is seen, the inner and outer scleral borders are poorly defined. 10 MHz ultrasonography shows an irregularly shaped tumor > 16 mm in diameter.
10 and 20 MHz Ultrasonography. High frequency ultrasound (UBM) reveals displacement of the iris root (arrow). Though no sclerostomy is seen, the inner and outer scleral borders are poorly defined. 10 MHz ultrasonography shows an irregularly shaped tumor > 16 mm in diameter.

This risks and benefits of observation, enucleation and radiation were discussed in detail. Primary enucleation was recommended and performed.

Comment

This case presents several classic findings in anterior uveal melanomas. Please share this case with your physicians in training. It is also important to note that not all ciliary body melanomas will exhibit these findings. Other findings of ciliary body melanomas include sector cataract and irregular astigmatism.

Lastly, there is some controversy about secondary radiation therapy as treatment for presumed residual microscopic orbital melanoma (due to extrascleral extension).

Without any compelling data to suggest its efficacy, and knowing the significant morbidity associated with 50 Gy (typical dose) of external beam radiation therapy to the anophthalmic orbit (dry socket, lash-brow loss, and mucus discharge), no additional treatment was given.

Alternatively, high dose rate intersitial radiation therapy can be used to allow for improved cosmetic results in cases where their is evidence of residual orbital melanoma.

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Case #9: Squamous Conjunctival Neoplasia

By Paul T. Finger, MD

History#1

1A man with a past ocular history of glaucoma (status-post trabeculectomy) and macular degeneration was referred to The New York Eye Cancer Center with squamous conjunctival neoplasia affecting his right eye.

One might imagine that he is at high risk for intraocular infiltration of his tumor.

Impression

Squamous Conjunctival Neoplasia

High-frequency Ultrasonography reveals the open sclerostomy (black arrow) and the overlying low-reflective tumor (white arrow). Note that the ciliary body is highly reflective (unlike the tumor). A multicystic bleb is noted on transverse section. High-frequency ultrasound obtained with the 20 MHz probe.
High-frequency Ultrasonography reveals the open sclerostomy (black arrow) and the overlying low-reflective tumor (white arrow). Note that the ciliary body is highly reflective (unlike the tumor). A multicystic bleb is noted on transverse section. High-frequency ultrasound obtained with the 20 MHz probe.

Note: With NO evidence of intraocular invasion, this patient was treated by tumor resection, superficial keratectomy, and adjuvant cryotherapy (to the episclera and conjunctival margins).

Comment: This case presents an unusual case of squamous conjunctival neoplasia that had grown over a functioning filtering bleb.

Findings on high-frequency ultrasound and the presence of iris neovascularization can be helpful in determining if squamous conjunctival neoplasia has infiltrated the eye.

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Case #8: 3D Ultrasound Topography of Choroidal Melanoma

By Paul T. Finger, MD

History

A View From Above The Tumor as it Regresses. Note that both the basal and apical dimensions shrink over time. Instead of 3 standard measurements (length, width and height) hundreds of surface data points are evaluated.
A View From Above The Tumor as it Regresses. Note that both the basal and apical dimensions shrink over time. Instead of 3 standard measurements (length, width and height) hundreds of surface data points are evaluated.

A male with a choroidal melanoma underwent 103Pd (palladium-103) ophthalmic plaque radiation therapy. Three dimensional (3D) ultrasound scans were taken immediately after, 3 and 7 months after treatment. The following renderings were made from that 3D data ultilizing a commercially available 3D ultrasound system.

Clinical Impression

Regressing Choroidal Melanoma

*Note* This patient was treated with palladium-103 ophthalmic plaque radiation therapy.

Comment

An Oblique View of the Tumor as it Regresses. A view from the side of the same tumor. Note that the tumor volume decreases over time. Hundreds of surface data points are evaluated.
An Oblique View of the Tumor as it Regresses. A view from the side of the same tumor. Note that the tumor volume decreases over time. Hundreds of surface data points are evaluated.

This case clearly demonstrates that choroidal melanomas regress in thickness throughout their volumes. Even though residual pigment may mask regression of the tumors width, 3D topographies clearly demonstrate that these areas of tumor become thinner (as does the tumor’s apex). Topographies should be availabe to evaluate and monitor tumor growth and regression.

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Case #7: Periocular Hydrocystoma

By Paul T. Finger, MD

History

A variably pigmented tumor of the caruncle was noted to grow over a 6-month period. Note the hyper-vascularity over the apex and subconjunctival pigment deposits (arrows).
A variably pigmented tumor of the caruncle was noted to grow over a 6-month period. Note the hyper-vascularity over the apex and subconjunctival pigment deposits (arrows).

A 71 year-old male presents with a variably pigmented and growing caruncular tumor. Subconjunctival melanocytic cysts were noted (arrows).

Clinical Impression

Hidrocystoma of the Caruncle

*Note* Due to progressive growth, hypervascularity, and subconjunctival pigmentation, this tumor was treated by excisional biopsy.

Comment

In this case hidrocystoma of the caruncle was suspected by clinical examination. High frequency ultrasound supported this diagnosis. Due to the presence of subconjunctival pigmentation and history of growth, an excisional biopsy was requested and performed. This case demonstrates the utility of high frequency ultrasound in the evaluation of the caruncle.

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Case #6: Giant Conjunctival Inclusion Cysts

By Paul T. Finger, MD

History

A digital slit-lamp photograph of a pigmented conjunctival tumor in the superonasal quadrant. The anterior, superior, inferior, and posterior margins are visible. The episcleral margin is obscured by the tumor.
A digital slit-lamp photograph of a pigmented conjunctival tumor in the superonasal quadrant. The anterior, superior, inferior, and posterior margins are visible. The episcleral margin is obscured by the tumor.

Two subconjunctival masses appearing 45 (Case 1) and 7 (Case 2) years after surgery.

Impression

Giant Conjuctival Inclusion Cysts

Comment

Conjunctival cysts typically cause cosmetic defects and Case 1, prevented proper eyelid closure. Since most are excised, high frequency ultrasound can help define the tumors extent. In Case 2, it differentiated between a solid and cystic pigmented conjunctival tumor.

Case 1: High-frequency ultrasound revealed that the lateral rectus muscle was not incarcerated within the tumor and defined the posterior tumor margin. The sclera was found to be intact. This was expected, but not known by slit-lamp examination. These findings were helpful for pre-operative planning.

A digital slit-lamp photograph of a conjunctival cyst overlying the insertion of the lateral rectus muscle. The anterior, superior and inferior margins are clearly visible. The posterior and episcleral margins are not visible. There is also a question as to the involvement of the lateral rectus muscle.
A digital slit-lamp photograph of a conjunctival cyst overlying the insertion of the lateral rectus muscle. The anterior, superior and inferior margins are clearly visible. The posterior and episcleral margins are not visible. There is also a question as to the involvement of the lateral rectus muscle.

Case 2: This tumor was more ominous looking. It was cystic, pigmented and close to the lacrimal gland. Again, high-frequency ultrasound was used to delineate the tumor’s edges, confirm scleral integrity, and that it was a cyst.

These cases were presented to aid in your continued care of patients with conjunctival cysts. Next time you plan excision of a large conjunctival cyst, you may want to consider evaluating it with a high-frequency ultrasound.

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Case #5: Lighting and a Choroidal Nevus

By Paul T. Finger, MD

History

This series of fundus photographs were taken during her consultation but at different levels of illumination.
This series of fundus photographs were taken during her consultation but at different levels of illumination.

In April of 2001, a 50-year-old female was referred for evaluation of a pigmented tumor in her right eye.

Unlike a choroidal melanoma this choroidal nevus did not have orange pigment, subretinal fluid or thickness greater than 2 mm. There were drusen over the area of pigmentation.

Impression

Choroidal Nevus

Comment

In cases of choroidal nevi, it is important to take a fundus photograph which serves as a baseline for future comparison. In this case the borders were clearly defined and a copy of this photograph was sent to the referring physician (to aid in his continued care). Should he repeat this fundus photograph, it will be important to compare the relative intraocular illumination used during photography. Consider the following series of fundus photographs taken during this patient’s initial visit.

We present this clinical pearl to aid in your continued care of patients with choroidal nevi. Next time you are evaluating a fundus photograph and think there is tumor growth, double check the background illumination to make sure the two pictures were taken at similar settings.

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Case #4: Iris Melanoma

By Paul T. Finger, MD

History

Though relatively small, this tumor caused ectropion uveae, pigment liberation, and sector cataract.
Though relatively small, this tumor caused ectropion uveae, pigment liberation, and sector cataract.

A 79-year-old female with a past history of hypertension and emphysema was referred for preoperative evaluation of a pigmented iris tumor in an eye with a 20/80 cataract OS.

At the The New York Eye Cancer Center her iris tumor was noted to exhibit ectropion uveae, pigment liberation into the angle, and sector cataract. Intraocular pressure measurements were 17 OU.

Indications for removal of presumed iris melanomas include: growth and secondary glaucoma. In this case there was neither. There is some controversy as to whether to remove or biopsy such lesions at the time of cataract surgery. After a detailed discussion of the potential risks and benefits of sector iridectomy, we decided to remove this tumor at the time of cataract surgery.

Plan:

A combined sector iridectomy, cataract extraction, and IOL insertion through a clear corneal incision.

Impression:

Malignant melanoma of the iris: Completely excised by histopathologic evaluation.

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Retinoblastoma Awareness Week: Common Childhood Eye Cancer Often Goes Undiagnosed

Data released Monday to coincide with World Retinoblastoma Awareness Week (May 8-15) reveals a troubling lack awareness of this deadly form of eye cancer.

Retinoblastoma is the most common eye cancer in young children. If not detected early, it’s also the most life-threatening. Each year, doctors diagnose more than 5,000 new cases of retinoblastoma. If caught early, this form of eye cancer is treatable and the eye can usually be saved. But unfortunately, awareness of this disease is low, and it often goes undiagnosed for long periods of time.

eyes retino
A recent survey conducted by the Childhood Eye Cancer Trust found that only 28% of parents had ever taken their children ages six and younger to an optician, general practitioner, or other health professional for an eye check. Half of the parents surveyed weren’t even aware that children can develop eye cancer.

Patrick Tonks, chief executive of CHECT, said it is imperative to increase awareness among both parents and physicians.

“For too long retinoblastoma has been shrouded in darkness – children are left in the dark because some or all of their vision has been stolen and parents and healthcare professionals are in the dark because of a lack of awareness.”

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Delay in diagnosing retinoblastoma can lead to the loss of an eye, or even death. Parents should always trust their instincts and there are several symptoms they should be aware of. These include unusual squinting, changes in the color of the iris, and painless redness or inflammation of eye. White reflections in the eye will also sometimes show up in photos taken with a flash camera.

It’s not only important for parents to be aware of retinoblastoma. Physicians should always address parental concerns relating to eye health and be sure to follow up on any symptoms. It is also recommended that doctors perform a red reflex test following symptoms such as squint or leukocoria to rule out retinoblastoma.

Simple awareness and follow-through can save a child’s vision, or even her life.

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