Sir,
A 75-year-old Japanese man was referred to us for evaluation of a brownish-black plaque of his left eyelid. He had been aware of the lesion for about 2 months. Physical examination showed a 5 × 5 mm, large, brownish-black plaque on the left epicanthic fold of the eyelid. The tumour had expanded into the palpebral and bulbar conjunctiva [Figure 1a]. A biopsy from the lesion revealed proliferation of atypical melanocytes with enlarged nuclei in the mildly thickened epidermis [Figure 1b]. A diagnosis of conjunctival melanoma was made. No obvious regional lymphadenopathy or orbital bone invasion was observed on computerised tomography. We performed en bloc orbital exenteration including the inferior nasal dorsum [Figure 1c]. In the resected specimen, we observed infiltration of mildly atypical melanocytes in the epithelium of the nasolacrimal duct [Figure 1d]. Immunohistochemically, the melanocytes were positive for both S-100 protein and melan A [Figure 1e]. No recurrence has been observed for 8 years after the surgery.
Figure 1.
(a) A brownish-black nodule on the left eyelid. (b) Proliferation of atypical melanocytes with enlarged nuclei in the mildly thickened epidermis (□ of a) (HE, ×100). (c) En bloc resected specimen including the inferior turbinate. (d) A few melanocytes observed in the epithelium of the nasolacrimal duct (□ of c) (HE, ×5). (e) Tumour cells in the nasolacrimal duct epithelium showing positive for melan A (□ of d) (×100)
Conjunctival melanoma is a very rare tumour accounting for about 5% of ocular melanomas and 0.25% of all melanomas.[1] Although targeted therapy and immune checkpoint inhibitors for melanoma have been developed,[2] conjunctival melanoma still has the potential for becoming a deadly extraocular tumour. Conjunctival melanoma has a higher tendency for local recurrence and distant metastasis. Risk factors for recurrence include thickness of the primary tumour of >4 mm, incomplete excision at the time of surgery, non-limbal tumour location, superior quadrant location and corneal involvement of >2 mm.[1]
It has been reported that 0.8%–9.6% of cases of conjunctival melanoma infiltrate to the lacrimal drainage apparatus.[3] Raksha et al.[4] also reported a discontinuous nasolacrimal duct infiltration of conjunctival melanoma. They proposed the term ‘melanorrhoea’ for spreading of conjunctival melanoma by floating tumour cells in the tear film, causing indirect tumour extension into the nasolacrimal duct. Satchi et al.[5] reported five conjunctival melanomas with infiltration to the lacrimal drainage apparatus. They suggested surgical technique for orbital exenteration of the lacrimal sac, upper nasolacrimal duct and the lacrimal canaliculi in patients with conjunctival melanoma. However, there is no evidence that removal of orbital contents improves life outcome.
Currently, conjunctival melanoma is commonly managed with globe-sparing treatments such as local excision combined with topical chemotherapy, cryotherapy, radiotherapy or immunotherapy.
Our patient had a discontinuous nasolacrimal duct infiltration up to near the inferior nasal duct. The atypia of the infiltrating melanocytes was extremely mild, and we required immunostaining to confirm the diagnosis. It has been reported that two out of five cases recurred in the lacrimal sac a few years after exenteration.[5] Thus, the lacrimal drainage apparatus is a high-risk tissue for metastasis and recurrence as it is exposed to free tumour cells due to melanorrhoea. Visual function preservation is very important in treatment of conjunctival melanomas. However, if dissemination of the lacrimal drainage apparatus is suspected, en bloc resection including the inferior nasolacrimal duct should be considered.
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The authors certify that they have obtained all appropriate patient consent forms. The form the patient has given includes his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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