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Mrinali Patel, Kathryn Colby; Clinicopathological Features of Young Patients Diagnosed with Primary Acquired Melanosis with Atypia or Conjunctival Melanoma. Invest. Ophthalmol. Vis. Sci. 2012;53(14):4946.
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Conjunctival melanoma and primary acquired melanosis (PAM) with atypia are diseases of primarily middle-aged and elderly persons. This descriptive study describes the clinical features of young patients diagnosed with these conditions.
Patients of age 30 years or younger who were evaluated at Massachussetts Eye and Ear Infirmary between 1995 and 2011 with biopsy-proven diagnoses of conjunctival melanoma or primary acquired melanosis with atypia were identified. Their charts, operative reports, and pathology reports were reviewed.
Seven cases met the inclusion criteria above. The average age at diagnosis was 26.2 years (range 19 - 30 years). Four (57%) were female and three (43%) were male. Six (86%) were Caucasian and one (14%) was African American. Average followup was 4 years (range 1.13 months - 9.3 years). Prior to diagnosis, four (57%) had a history of a pigmented (43%) or erythematous (14%) conjunctival lesion. In addition, in one (14%) patient, the conjunctival lesion was likely a metastasis from an ipsilateral lower lid cutaneous melanoma metastastic to the lymph nodes. The lesions were located temporally in three (43%) patients and nasally, superiorly, and inferiorly in one patient each (14%). In one (14%) the lesion was diffuse, involving the temporal conjunctiva and spreading superior and inferiorly below the lids and to the fornices. Four (57%) cases exhibited corneal extension. The lesions were pigmented in five (71%) patients, and amelanotic with only scant pigment in two (29%). Six (86%) lesions were elevated, three (43%) exhibited feeder vessels, and none (0%) possessed cystic features. All cases underwent biopsies with or without epithelial curettage, cryotherapy, and/or topical mitomycin-C. On pathology, five (71%) were diagnosed as PAM with atypia and two (29%) were diagnosed as malignant melanoma, one arising from a conjunctival nevus and another likely representing a metastasis from cutaneous melanoma. Neither patient with melanoma developed recurrences after excision. Three (60%) of the five patients initially diagnosed with PAM with atypia developed recurrent lesions with malignant transformation to melanoma, within one to seven years. One of these patients also developed widespread metastases.
PAM with atypia and conjunctival melanoma can occur in young patients and are associated with a risk of recurrence and metastasis, similar to older patients with these conditions.
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