Conjunctival Melanoma


Conjunctival Melanoma Presenting as Pigmented Patch
Clinical image shows an expansile pigmented patch image at the limbus image and adjacent to the iris image. Bulbar conjunctiva close to the limbus is the most common location for primary acquired melanosis (PAM).



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Scanning Magnification of PAM With Atypia
Scanning magnification of this conjunctival shave biopsy shows a lentiginous basilar proliferation image that stands out even at the this power. The radial extension appears to be extensive.

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Higher Magnification of PAM With Atypia
Higher magnification of the same case confirms that atypical melanocytes image have replaced the basilar keratinocytes in the conjunctiva. There is some pagetoid upward scatter of melanocytes image.

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Higher Magnification Shows Nuclear Hyperchromasia and Pleomorphism
High-power magnification shows poorly nested proliferation of atypical melanocytes in the same lesion of PAM with atypia. The upward scatter image, nuclear hyperchromasia image, and nuclear pleomorphism resembles cutaneous melanoma in situ.


TERMINOLOGY


Abbreviations




• Conjunctival melanoma (CM)


Synonyms




• Conjunctival malignant melanoma


Definitions




• Malignant proliferation of conjunctival melanocytes commonly associated with primary acquired melanosis (PAM) with atypia


CLINICAL ISSUES


Epidemiology




• Incidence
image Rare; accounts for only 2-3% of ocular cancers

image Accounts for 1% of noncutaneous malignant melanoma

• Age
image More common in older individuals (mean age at presentation: 50-60 years)

image Exceedingly rare in children younger than 15 years of age (only 28 reported cases)


Presentation




• Asymptomatic raised, pigmented plaque, macule, or tumor

• Can range in size from millimeters to large tumor masses

• Bulbar conjunctiva close to limbus is most common location


Natural History




• Majority of cases (53-75%) arise in setting of PAM with atypia

• Remainder (18-30%) arise de novo

• Minority of cases (5%) are associated with preexisting melanocytic nevus


Treatment




• Options, risks, complications
image Avoiding manipulation of tumor during surgery reduces local recurrence rate and lymphatic spread

• Surgical approaches
image All should be completely excised with 2- to 3-mm tumor-free margins

image Sentinel lymph node biopsy recommended for high-risk tumors
– > 10 mm in diameter and > 2 mm in thickness

– Nonlimbus locations

• Adjuvant therapy
image Risk of recurrence is reduced with adjuvant cryotherapy, irradiation, or topical chemotherapy


Prognosis




• Conjunctival melanoma arising in association with PAM has higher risk of local recurrence
• 5-year survival rate: 87-95%

• 10-year survival rate: 70-86%

• After 10 years, 50% of tumors will recur locally and about 25% will metastasize

• Adverse clinical prognostic indicators include

image Nonbulbar (fornix, palpebral) location

image Involvement of lymphatics-rich caruncle

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Conjunctival Melanoma

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