Case #12: PAM With Atypia

By Paul T. Finger, MD

Impression

Pigmented tumor could be seen involving the plica and inferior fornix.
Pigmented tumor could be seen involving the plica and inferior fornix. Extensive involvement of the lower eyelid suggests diffuse involvement of the conjunctiva (beyond the pigmented margins).

PAM with Atypia with Eyelid Extension.

*Note* This risks and benefits of observation, exenteration, excision with and without cryotherapy and/or topical mitomycin, as well as radiation were discussed with this patient in detail. Primary excision with subsequent topical mitomycin therapy was recommended and performed. The nodular and resectable areas were removed. Topical mitomycin was employed to address the residual diseased epithelium.

Comment

This case presents several classic findings of PAM as well as the unusual finding of extension onto the lower eyelid. Other findings of conjunctival melanomas and primary acquired melanosis include thickness, neovascularization, and corneal epithelial extension. Intraocular extension is very rare, but should this tumor recur, orbital invasion is not uncommon.

Your Questions or Comments

3 months after resection (conjunctival and eye lid)
These photographs were taken 3 months after resection (conjunctival and eye lid) followed by topical mitomycin chemotherapy. Despite this excellent result, this patient is at risk for recurrent melanoma.

Melanie Kazlas, M.D.
Director of Pediatric Ophthalmology
Boston Medical Center

Question: Yesterday I saw a 10 year old Hispanic/African American child for a routine visit. He had salt and pepper melanosis in the inferior fornix OS>OD as well as a flat pigmented lesion of the nasal inferior tarsal conjunctiva (2x2mm). I often see perilimbal melanosis in my darkly pigmented patients. Can my patient also be classified as having “racial” melanosis?

Answer: I too see a lot of racial melanosis. As a general rule, if it is bilateral, it is probably racial melanosis. As you have noted (unlike your case) racial melanosis primarily involves the bulbar conjunctiva. It is also important to know that nevi can grow during puberty.

In general, I photograph and follow most of these patients. Photography has been made relatively inexpensive and easier by digital technology. My threshold for biopsy of suspicious or growing lesions is low. Make sure an ophthalmic pathologist sees the specimen.

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Case #11: 3D Ultrasound of the Disorganized Eye

By Paul T. Finger, MD

History

[10 MHz] 3D Ultrasonography Reveals - *Note* Ultrasonography reveals an open funnel retinal detachment (white arrow). Note the echolucent vitreous humor. The posterior choroidal detachments (black arrows) exhibit variable internal reflectivity consistent with blood. A = anterior, S = superior, T = temporal.
[10 MHz] 3D Ultrasonography Reveals – *Note* Ultrasonography reveals an open funnel retinal detachment (white arrow). Note the echolucent vitreous humor. The posterior choroidal detachments (black arrows) exhibit variable internal reflectivity consistent with blood. A = anterior, S = superior, T = temporal.
3D ultrasound is particularly helpful in evaluation of the disorganized eye. This case of retinal detachment with hemorrhagic choroidals illustrates this advantages of 3D ophthalmic ultrasonography. This 60 year old male hypertensive patient was referred with neovascular glaucoma. Both 2D and 3D ultrasonography were performed.

Unlike static 2D images, a 3D volume can be rotated, oriented, and sectioned. This movie allows the viewer to review 5 (out of hundreds of possible) sections of this 3D ocular volume.

The original 3D block was composed of 90 sequentially acquired 2D images reconstructed into a 9.5 megabyte file. Due to limitations of the Internet, the entire file (volume) is not displayed. While evaluating this animation, you are experiencing a retrospective review of the ultrasound examination and realizing the potential of telemedicine.

Impression

Open Funnel Detachment and Hemorrhagic Choroidals

Comment

This case presents two of the many unique capabilities of 3D ultrasonography:

  1. The ability to quarter the acquired volume and thereby image 3 planes (3 dimensions) simultaneously.
  2. The ability to replay the examination in a remote location (as you are doing in limited form). This capability will allow ultrasound specialists to review and comment on examinations performed in remote locations.

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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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