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CLINICAL MANAGEMENT GUIDELINES
Conjunctival pigmented lesions
Aetiology
Conjunctival pigmented lesions include a spectrum of benign, premalignant and malignant melanocytic conditions:
Melanosis
 Hypermelanosis (i.e. melanin overproduction by normal
melanocytes)
- racial melanosis
- secondary melanosis (Addison’s disease; conjunctival
lesions)
 Primary Acquired Melanosis (PAM), also known as Conjunctival
Melanocytic Intraepithelial Neoplasia (C-MIN)
- with or without ‘atypia’ (cellular structural abnormalities),
graded according to cytomorphology, melanocytic density and
spread to superficial layers of epithelium. Severe disease
amounts to Melanoma In Situ (i.e. confined to epithelium)
 Congenital Melanocytosis
- hyperpigmentation of episclera as a result of an overpopulation of melanocytes, also occurring in uvea and skin
(i.e. Naevus of Ota). Predisposes to melanoma
Naevus
 the most common conjunctival pigmented lesion: 52% of ocular
pigmented lesions
 congenital or acquired
 cluster of naevus cells in the conjunctival epithelium, usually
extending to substantia propria. Cysts are often present
Melanoma
 rare malignant tumour arising from naevus, PAM or de novo: 3-5%
of ocular malignancies, incidence <1 per million of population per
year
 metastasises to regional lymph nodes and systemically, especially
if involving caruncle and/or non-bulbar conjunctiva
Systemic disorders and drugs linked rarely with conjunctival
pigmentation include:
 Addison’s disease (adrenal gland insufficiency)
 alkaptonuria (congenital enzyme deficiency)
 drugs (chlorpromazine, topical epinephrine, etc.)
Predisposing factors
Symptoms
Signs
Some of these conditions are illustrated at :
http://www.eyecancer.com/research/image-gallery/3/conjunctival-tumors
Epithelial melanosis is common in dark-skinned ethnicities.
PAM typically affects older white-skinned patients (rarely in darkskinned)
Melanoma is more common in people with fair skin and blue eyes,
extremely rare in dark-skinned races. Presentation peaks in mid-fifties
Asymptomatic except for cosmetic concern
Ethnic melanosis
Bilateral, asymmetrical, flat, intra-epithelial (moves freely over sclera),
patchy, brown pigmentation, most prominent in palpebral aperture
especially at limbus or where anterior ciliary arteries perforate the sclera,
Conjunctival pigmented lesions
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Date of search 17.02.16; Date of revision 13.06.16; Date of publication 17.10.16; Date for review 16.02.18
© College of Optometrists
CLINICAL MANAGEMENT GUIDELINES
Conjunctival pigmented lesions
develops in early years (static by adulthood)
C-MIN/PAM
Unilateral, any part of conjunctiva (including tarsal or forniceal), flat, intraepithelial (moves freely over sclera), single or multiple, indistinct areas,
light to dark brown, no cystic spaces, often extensive, can be stable or
may change (enlarge, shrink, darken or lighten)
Congenital Melanocytosis
 ocular
Multifocal, slate-grey or blue grey, sub-epithelial (does not move
freely over sclera)
 dermal
Mottled, blue to purple, discolouration of skin around the eye
Naevus
Solitary, sharply-demarcated, flat or slightly-elevated, intra-epithelial
(moves freely over sclera). Confined to interpalpebral zone (very rare in
palpebral or forniceal conjunctiva), most commonly adjacent to but not
touching the limbus.(less frequently at plica, caruncle, lid margin). NB: a
pigmented lesion that straddles the peripheral cornea is highly
suspicious and should be assumed to be a malignant melanoma.
Presents in second or third decade when naevus becomes more heavily
pigmented. Colour ranges from deep brown through pink to barely
perceptible pigment. Often contains cystic spaces. Very rarely
vascularised or inflamed
Melanoma
Nodular, well-vascularised mass with large conjunctival feeder vessels,
fixed to underlying sclera (assess the degree of tethering, under topical
anaesthesia). May be pigmented or non-pigmented (amelanotic), and
nodular, diffuse or mixed
Differential diagnosis
Conjunctival intraepithelial squamous neoplasia (i.e. carcinoma in situ)
can resemble an amelanotic melanoma
Management by Optometrist
Practitioners should recognise their limitations and where necessary seek further advice or refer
the patient elsewhere
Non pharmacological
Ethnic melanosis
Has no malignancy potential and requires no treatment
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
PAM / C-MIN
Sometimes has potential for malignancy (up to 13%) – refer for
assessment, which requires biopsy to identify atypia
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
Congenital Ocular Melanocytosis
Has potential for malignancy – refer.
Is associated with malignant melanoma of the affected skin, orbit and
uveal tract (fundoscopy with pupillary dilatation is required).
Also associated with hyperpigmentation elsewhere in the eye including
the trabeculum (regular monitoring for glaucoma required)
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
Conjunctival pigmented lesions
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Date of search 17.02.16; Date of revision 13.06.16; Date of publication 17.10.16; Date for review 16.02.18
© College of Optometrists
CLINICAL MANAGEMENT GUIDELINES
Conjunctival pigmented lesions
Naevus
Generally requires no treatment, but very rarely progresses to a
malignant melanoma. Advise patient to report any increase in size,
elevation or colour. Review after 6 months and then every 12 months if
lesion unaltered. Photo-documentation strongly advised
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
Melanoma
Refer urgently (potentially sight– and life– threatening).
Disseminates by local extension and by spread via lymphatic system
(check preauricular and submandibular lymph nodes)
(GRADE*: Level of evidence=low, Strength of recommendation=strong)
Pharmacological
Management Category
None
B1: Routine referral to ophthalmologist
 PAM /C-MIN
 Naevus, especially if non-bulbar conjunctiva is involved
 Congenital Ocular Melanocytosis
B2: Alleviation/palliation: normally no referral to ophthalmologist
 Mild ethnic melanosis
A3: Urgent (within one week) referral to ophthalmologist
 Melanoma
Possible management by Ophthalmologist
PAM requires multiple biopsies to detect the histopathological
characteristics that predict invasive melanoma.
Tests for malignancy and excision where required
Melanoma is usually treated by en bloc surgical excision with adjuvant
therapy such as: mitomycin C, radiotherapy, topical chemotherapy.
More than 50% develop local tumour recurrence with 20% requiring
orbital exenteration and 20-30% developing fatal metastases
Evidence base
*GRADE: Grading of Recommendations Assessment, Development and
Evaluation (see http://gradeworkinggroup.org/toolbox/index.htm)
Sources of evidence
Damato B, Coupland SE. Management of conjunctival melanoma. Expert
Rev Anticancer Ther. 2009;9(9):1227-39
Harooni H, Schoenfield LR, Singh AD. Current appraisal of conjunctival
melanocytic tumors: classification and treatment. Future Oncol.
2011;7(3):435-46
Levecq L, De Potter P, Jamart J. Conjunctival Nevi, Clinical Features
and Therapeutic Outcomes. Ophthalmology 2010;117:35-40
LAY SUMMARY
The conjunctiva (the transparent skin over the white of the eye) sometimes develops brown
discolouration. This is classified according to the cause:
Conjunctival pigmented lesions
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CLINICAL MANAGEMENT GUIDELINES
Conjunctival pigmented lesions
Hypermelanosis: melanocytes (the cells of the body that produce the dark pigment melanin) go into
overproduction. This may be a normal characteristic of dark-skinned races, or it may be caused by
disease elsewhere in the body.
Primary Acquired Melanosis: unusually large numbers of melanocytes develop. This is rare in
dark-skinned races and tends to affect older white-skinned people.
Congenital Melanocytosis: similar, except present from birth.
A Naevus, that is a brown spot on the conjunctiva, may be present from birth or may arise later.
This is the commonest of all the conjunctival pigmented lesions. Usually it does not grow or
spread.
Sometimes, a naevus changes into an Invasive Melanoma, also known as a Malignant Melanoma,
which can spread to other parts of the body. This particularly affects people with fair complexions
and is seen only very rarely in dark-skinned people.
There are also some uncommon generalised diseases that may produce discolouration of the
conjunctiva. Some prescription drugs may also cause a similar effect.
Depending on the nature of the pigmented lesion, optometrists may monitor the condition
themselves, or refer to an ophthalmologist routinely or urgently. Mild ethnic hypermelanosis does
not need to be referred.
The ophthalmologist will carry out tests to identify which condition the patient has. Melanoma is
usually treated with surgery and additional drug therapy. Careful follow-up is required.
Conjunctival pigmented lesions
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Date of search 17.02.16; Date of revision 13.06.16; Date of publication 17.10.16; Date for review 16.02.18
© College of Optometrists