Primary acquired melanosis with atypia (biopsy proven).
Malignant melanoma can occur on the surface of the eye (conjunctiva and cornea). It can start on its own, as a pre-existing nevus or arise within newly formed pigmentation.
Symptoms
Most patients notice either a nodule forming on, or a darkening of the surface of the eye. Large tumors can bleed resulting in “bloody tears.” The tumor can extend onto the eyelid skin and lymph nodes in front of the ear (preauricular) or neck (cervical). Involved lymph nodes enlarge and can be felt (palpable) during examination.
Diagnosis
The doctor will examine your eyes. This includes an examination of all the conjunctival surfaces (including the inside of the eyelids). Pigmented conjunctival tumors are considered suspicious if they have large blood vessels running toward them, if they extend onto the cornea or if they extend into the conjunctival fornices.
Photographs should be taken of all the conjunctival and corneal surfaces at the time of initial examination (prior to any biopsy). This is important because it helps the doctor document the extent of the condition (stage the tumor) and can be used to plan for surgery and aid in follow up. Removing a conjunctival melanoma prior photography and/or referral to an eye cancer specialist can decrease the patients chance for cure.
Biopsy can determine whether a pigmented conjunctival tumor is a nevus, primary acquired melanosis, or conjunctival melanoma. A conjunctival nevus and benign acquired melanosis can be photographed and followed for evidence of change prior to intervention. Malignant conjunctival melanoma and intra-epithelial melanoma (in situ) should be removed or destroyed.
*Note* In a multicenter international effort to develop a staging system for conjunctival melanoma, the authors agreed that the term Primary acquired melanosis with atypia should be abandoned in favor of conjunctival melanoma in situ.
Treatments
Most very small areas of conjunctival hyperpigmentation can be photographed and followed for evidence of growth or change prior to treatment. If they are raised, hypervascular or extend onto the cornea a biopsy is reasonable. Small tumors can be completely removed, and if they are found to be malignant additional cryotherapy (freezing) or adjuvant topical chemotherapy may be necessary.
Melanomas can be difficult to treat if they occur at multiple spots on the eye. In these cases, surgical removal with freezing therapy may not control this tumor. Then chemotherapy eye-drops can be used for patients with conjunctival melanoma. Chemotherapy eye drops treat the entire surface of the eye, and is less dependent upon defining the tumors edges.
This epibulbar dermolipoma is found to infiltrate the peripheral cornea and extend back to the lacrimal gland.
Epibulbar dermoids are benign tumors. They tend to be firm, white-yellow or pinkish tumors straddling the limbus in the temporal (primarily inferotemporal) quadrants. They are located both over the cornea and sclera. They can range from several millimeters to over a centimeter in size. They are typically unilateral (found on one eye), but can be bilateral.
Dermoids are choristomas (normal tissues that are in the wrong place). Made up of cutaneous and subcutaneous tissue, it is not uncommon for dermoids to contain hair and other skin structures. These tumors can be found on the eye, adnexa and orbit.
Dermolipomas are more commonly found in the superotemporal quadrant extending to the lacrimal gland and/or orbit.
Symptoms
Most patients with epibulbar dermoid or dermolipomas have no symptoms unless hairs or other dermal structures cause local irritation. The lesions do cause a cosmetic defect.
Diagnosis
The diagnosis of dermoid and dermolipoma is made by ophthalmic examination. These lesions are typically present at birth and do not progress. Though ultrasound and radiographic imaging may be required to investigate the extent of the tumor, biopsy is not necessary.
Dermoids or dermolipomas are more likely to be associated with Goldenhar’s Syndrome if they are multiple or bilateral. Goldenhar Syndrome is associated with dermoid tumors at the tragus of the ear and facial dysostosis.
Treatments
It is very important to make sure your child does not have a secondary astigmatism related to corneal tumor involvement. Early treatment of astigmatism can prevent amblyopia (loss of vision).
Surgery can be performed to limit the cosmetic defect, but there are many reports of secondary complications related to thinning of the scleral “eye wall” and corneal astigmatism.
Surgical removal of dermolipomas (that can extend into the lacrimal gland and orbit) can be associated with lacrimal gland dysfunction (dry eye) and double vision. Care must be taken to preserve the overlying conjunctiva and lacrimal gland.
Additional Info
Dermoids and dermolipomas can be associated with Goldenhar Syndrome or Linear Nevus Sebaceous Syndrome.
Malignant cancers can grow on the surface of the eye. They usually start from the membrane that covers most of the eye called the conjunctiva. The most common conjunctival cancers are squamous carcinoma, malignant melanoma and lymphoma.
Malignant melanoma used to be called PAM with atypia, but the newest AJCC staging system authors suggest we stop using the term PAM with atypia and call it melanoma in situ. This was because PAM with atypia doesn’t define it as a cancer. Pathologists call these specimens Tumor in situ or Tis.
Squamous carcinoma of the conjunctiva can form a nodule or diffusely spread out over the surface of the eye. Only very large squamous conjunctival cancers and those in patients who are immunosuppressed metastasize to other parts of the body. But they can invade into and around the eye, into the orbit and sinuses.
Malignant melanoma can start as a conjunctival nevus, arise as newly formed pigmentation (or variably pigmented) within the conjunctiva or onto the cornea. A simple biopsy can determine whether a pigmented conjunctival tumor is a benign nevus, primary acquired melanosis, or conjunctival melanoma.
Lymphoma can also occur in the conjunctiva. These tumors look like red or salmon-colored patches on the eye and can be the first sign of systemic lymphoma. Eye cancer specialists usually biopsy lymphoid tumors so that a pathologist can perform special immunologic and genetic studies on the tumor cells. These techniques are used to determine if the tumor is benign or malignant. Patients with lymphoid conjunctival tumors should have a complete medical check up and be examined by a hematologist-oncologist. Other “less common” conjunctival cancers are reviewed in this section.
Symptoms
Most conjunctival tumors do not cause symptoms. Patients typically seek medical attention because they notice a discoloration on the eye or extension of the tumor onto the cornea. Conjunctival tumors can also be found by an eye care specialist during a routine eye examination.
Diagnosis
Most small benign-appearing conjunctival tumors can be photographed and followed for evidence of growth prior to biopsy or treatment. If they are raised, hypervascular or extend onto the cornea a biopsy is more reasonable. At The New York Eye Cancer Center we typically obtain an office-based cytology specimen for squamous carcinoma. This lets us know the tumor is squamous and avoids a trip to the operating room. In contrast, both melanoma and lymphoma require more histopathology and special pathology analysis. Therefore, those tumors require surgical biopsy or if small, excision.
Conjunctival melanomas require special treatment. The natural history of these tumors has been characterized as presenting with multiple tumors or non-pigmented skip areas.
Therefore, it is difficult and some think impossible to define the extent of the tumor on clinical examination.
The So-Called “No Touch Technique”
The natural skip areas of pigmentation, multifocal presentation and high rates of recurrence have led some eye cancer specialists think that handling conjunctival melanoma promotes spread. They fear that tumor cells get stuck on the instruments that if reused, implant tumor on other, unaffected parts of the globe.
However, Dr. Finger disagrees with this theory and its so called “no touch technique” that requires surgeons to get new instruments after each time they touch the tumor (to prevent transplantation). However, there are no other cancers where surgeon-related transplantation has been found. More likely, these eye cancer specialists operate on one area, thinking that the pigmented portion of the tumor is the complete extent of disease. Unaware, there are other non-pigmented or small tumors that are not yet visible and later grow. Then they think they transplanted the tumor.
At The New York Eye Cancer Center, when conjunctival melanoma is suspected, Dr. Finger utilizes his specially designed cryotherapy devices “Finger-tip” cryotherapy probes” to make sure the tumor and a surround of normal appearing tissue is treated before he touches the tumor. Then the tumor can be safely removed without fear of transporting tumor cells or invaginating the edges of the wound rendering tumor too deep to treat with chemotherapy eye drops. At The New York Eye Cancer Center, topical chemotherapy eye drops are used for treatment of both squamous and melanoma cancers. These drops have been found to reduce or more commonly eliminate the need for extensive surgery.
Evaluation of the biopsy specimen should be performed by an experienced ophthalmic pathologist. If there isn’t an ophthalmic pathologist in your area, you can request that the histopathology slides be sent for second opinion.
Treatment: General Guidelines
Small tumors can be completely removed, and if they are found to be either squamous carcinoma or malignant melanoma, additional cryotherapy (freezing) is likely to improve local tumor destruction and thus prevent recurrence. Dr. Finger has developed specialized “Finger-tip” cryotherapy probes to uniformly freeze large surfaces of the eye with minimal intraocular penetration (see innovations section).
Chemotherapy Eye Drops:
Conjunctival melanoma and squamous carcinoma can be difficult to treat if they are “mulitfocal” – occur in multiple spots on the eye. In these cases, even surgical removal with freezing therapy may not control the tumor. Dr. Finger has found that “Chemotherapy eye-drops can be used for and are often better than surgery for most patients with conjunctival cancers.” Chemotherapy eye-drops treat the entire surface of the eye, are less dependent upon defining the tumors edges, and decreases the chance of scarring (symblepharon) after surgery. Researchers at The New York Eye Cancer Center recently published on treatment of “Giant Conjunctival Squamous Carcinoma’ with chemotherapy eye drops alone (no surgery).
Systemic lymphoma can usually be treated with standard chemotherapy that is also likely to cure malignant ocular lymphomas. If the eye is the only site of malignant lymphoma, low dose external beam radiation therapy is commonly employed.
"Very well treated by Dr. Finger. He explained everything I needed to know about my issue with detail and attention, putting me at ease and giving me confidence to handle this problem for the rest of my life.”
– N.N.
