RT Journal Article
SR Electronic
T1 Choroidal malignant melanoma in an albino.
JF British Journal of Ophthalmology
JO Br J Ophthalmol
FD BMJ Publishing Group Ltd.
SP 840
OP 845
DO 10.1136/bjo.73.10.840
VO 73
IS 10
A1 A G Casswell
A1 A C McCartney
A1 J L Hungerford
YR 1989
UL http://bjo.bmj.com/content/73/10/840.abstract
AB This is the first report of an amelanotic melanoma arising in the unpigmented choroid of a tyrosinase-positive oculocutaneous albino (TPOCA). Melanosomes within the tumour showed a maturation arrest in the unpigmented type II (premelanosome) phase. Other neural crest derived melanocytes in iris and choroid showed similar limited melanogenesis. The neuroectodermally derived melanocytes of the iris, ciliary body, and retinal pigment epithelium (RPE) contained mature melanosomes, though clinically the RPE was pale. The significance of this tumour arising in an albinotic eye is discussed.