Perspective
Conjunctival melanoma and melanosis: a reappraisal of
terminology, classification and staging
Bertil Damato MD PhD FRCOphth
1
and Sarah E Coupland MBBS PhD FRC Path
2
1
Ocular Oncology Service, Royal Liverpool University Hospital, and
2
Department of Pathology, School of Cancer Studies, University of
Liverpool, Liverpool, UK
ABSTRACT
This paper aims to stimulate debate on the terminology,
classification, grading and staging of conjunctival melanosis
and melanoma.We audited our results with 76 invasive con-
junctival melanomas. Staging according to the sixth edition of
the Tumour Node Metastasis (TNM) system did not corre-
late well with tumour extent and outcome. Approximately
50% of invasive melanomas were associated with ‘primary
acquired melanosis with atypia’, a term which in our opinion
underestimates the gravity of this disease. We also found
deficiencies in the grading, terminology and classification of
conjunctival melanocytic abnormalities. In summary, we
suggest that the term ‘primary acquired melanosis’ be
reserved for clinical diagnosis. Histologically, this abnormality
can be categorized more precisely as either ‘hypermelanosis’
or ‘conjunctival melanocytic intraepithelial neoplasia
(C-MIN)’. ‘Primary acquired melanosis without atypia’ can be
termed more accurately as ‘C-MIN without atypia’. In view of
the high risk of invasive melanoma, we suggest that ‘primary
acquired melanosis with atypia’ be termed ‘C-MIN’ with
atypia, with the more severe changes regarded as melanoma
in situ.To improve objectivity in the reporting of C-MIN, we
propose a scoring system based on horizontal and vertical
spread and degree of severity of melanocytic atypia. We
suggest that the TNM staging system for conjunctival
melanoma be revised to: (i) include a Tis stage; (ii) take
account of tumour size, quadrant and caruncular involve-
ment; and (iii) improve staging of any local invasion beyond
conjunctiva.
Key words: conjunctival neoplasm, disease-specific mortal-
ity, melanoma, ophthalmology, pathology.
INTRODUCTION
The terminology of conjunctival melanotic abnormalities is
confusing. For example, the noun, ‘melanosis’, which refers to
melanotic pigmentation visible to the naked eye, is used to
encompass both melanin hyper-secretion and melanocytic
proliferation.
1
Furthermore, there is disagreement as to
whether melanocytic intraepithelial neoplasia with atypia is
pre-cancerous or cancerous.
2,3
As a result, the same disease at
a particular point in the spectrum of malignancy may be
termed ‘primary acquired melanosis with atypia’ (PAM with
atypia) by one pathologist and ‘melanoma in situ’ by another,
possibly risking under-treatment or over-treatment.
Classification of conjunctival melanocytic disorders is
valuable to clinicians and histopathologists considering the
differential diagnosis of a particular case. Various systems
have been developed (see later text), but in our opinion,
these all have limitations, such as including congenital ocular
melanocytosis, which does not involve conjunctiva, being
sub-conjunctival.
4
The histological grading of PAM with atypia is variably
described using terms such as ‘mild’ and ‘severe’.
5
There is
scope for a system that grades this conjunctival melanocy-
tic intraepithelial neoplasia (C-MIN) more objectively and
reproducibly. This would make it easier to detect progression
when sequential biopsies are performed over a long period
and would enhance communication between the pathologist
and ophthalmologist. Such a scoring system would also facili-
tate multicentre collaboration when evaluating treatment.
Conjunctival melanomas are staged according to the
Tumour Node Metastasis (TNM) system, developed by the
American Joint Committee on Cancer and the Union Inter-
national Contre Cancer.
6
The objectives of this system are to
stage disease in a standardized fashion so as to enhance
prognostication, treatment planning and multicentre studies.
We feel that the sixth edition categorizes conjunctival mela-
nomas into stages that do not correlate adequately with
Correspondence: Dr Bertil Damato, Ocular Oncology Service, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK. Email:
bertil@damato.co.uk
Received 16 April 2008; accepted 3 October 2008.
Clinical and Experimental Ophthalmology 2008; 36: 786–795
doi: 10.1111/j.1442-9071.2008.01888.x
© 2008 The Authors
Journal compilation © 2008 Royal Australian and New Zealand College of Ophthalmologists