Primary choroidal melanoma in phakomatosis pigmentovascularis IIa.
OBJECTIVE
To describe a previously unreported association between phakomatosis pigmentovascularis (PPV) IIa and primary choroidal melanoma.


METHODS
Case series.


METHODS
Three patients with PPV type IIa and choroidal melanoma.


RESULTS
Nevus flammeus was present unilaterally in each patient, involving the hemiface, the hemithorax, or both. Ocular melanocytosis also was present unilaterally in all patients involving the sclera, the iris, the choroid, the distribution of the trigeminal nerve, or a combination thereof. Renal cysts were noted in patient 1, and a nevus anemicus of the neck was noted in patient 3. A unilateral choroidal melanoma was detected in each of these 3 patients, in each patient involving the same eye in which melanocytosis had been seen. No metastases were found in any patient.


CONCLUSIONS
Clinicians should be aware of the possibility that PPV, and in particular that occurring in patients with ocular melanocytosis, can be associated with choroidal melanoma.
Tran
Hoai Viet
HV
Jules Gonin University Eye Hospital, Lausanne, Switzerland.
Zografos
Leonidas
L
D000328:Adult; D002830:Choroid Neoplasms / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D019583:Dose Fractionation, Radiation; D005260:Female; D006801:Humans; D007429:Intraocular Pressure; D015811:Iris Neoplasms / Q000150:complications / Q000473:pathology / Q000532:radiotherapy; D008297:Male; D008545:Melanoma / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D008548:Melanosis / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D008875:Middle Aged; D009508:Nevus, Pigmented / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D019339:Port-Wine Stain / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D015422:Scleral Diseases / Q000150:complications / Q000473:pathology / Q000532:radiotherapy