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Diagnosis: Ocular Melanoma
Histopathology:
Conjunctival and subconjunctival tissue from the right inferior
fornix shows highly atypical melanocytes within the mucosa and
inflitrating into the underlying stroma (Fig4). Skin from the
right lower eyelid shows melanoma in situ, lentiginous type, with
atypical melanocytes within the epidermis (Fig5).
Discussion:
Though rare, ocular melanoma is the most common primary intraocular
malignancy in adults, with an overall annual incidence of less
than 10 per million per year. Approximately 85% of ocular melanomas
arise from the uveal tract (choroid, iris, ciliary body) and 5%
from the conjunctiva.
Conjunctival melanomas may arise from primary acquired melanosis
(PAM) with atypia, conjunctival nevi, or de novo. Up to 75% of
conjunctival melanomas are thought to arise from PAM, which is
a proliferation of melanocytes, usually unilateral, that presents
in middle age. When PAM is accompanied by atypia, there is a high
risk of malignant degeneration.
Conjunctival melanoma may metastasize in up to 30% of cases, most
commonly to regional lymph nodes, lung, brain, or liver. Sentinel
lymph node mapping is currently being studied in periocular melanoma.
Estimates of conjunctival melanoma-related death range from approximately
15-25% at 10 years.
Treatment:
Treatment options include excision followed by adjunctive cryotherapy,
radiotherapy, or topical chemotherapy for localized lesions; radiotherapy
alone for more extensive lesions; and orbital exenteration
for extensive, recurrent, and/or aggressive tumors.
Given the extensive nature of this patient's disease, preservation
of his right eye was not possible. He underwent an anterior exenteration
of the right orbit with removal of the globe, lids, and pigmented
tissue of the of the right cheek.
References:
1. Char DH. Tumors of the Eye and Ocular Adnexa. BC Decker Inc, London. 2001.
2. Damato A. Melanotic conjunctival lesions. In: Ocular tumours: Diagnosis and treatment. Butterworth-Heinemann, Boston. 1999.
3. Esmaeli B. 2000. Sentinel lymph node mapping for patients
with cutaneous and conjunctival malignant melanoma. Ophthal Plastic
Recons Surg 16: 170-172.
4. Shields CL et al. 2000. Conjunctival melanoma: risk factors
for recurrence, exenteration, metastasis, and death in 150 consecutive
patients. Arch Ophthalmol 118: 1497-1507.
5. Werschnik C and Lommatzsch PK. 2002. Long-term follow-up of patients with conjunctival melanoma. Am J Clin Oncol 25: 248-255.
