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Leucocoria as the presenting sign of a ciliary body melanoma in a child
  1. HAKAN DEMIRCI,
  2. CAROL L SHIELDS,
  3. JERRY A SHIELDS,
  4. SANTOSH G HONAVAR
  1. Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA
  2. Pathology Department
  1. RALPH C EAGLE, JR
  1. Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA
  2. Pathology Department
  1. Dr Carol L Shields, Oncology Service, Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA 19107, USA

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Editor,—Uveal melanoma is generally a disease of adulthood. It has been reported that 0.6% to 1.6% of all uveal melanomas occur in patients under 20 years of age. In a review of 3706 consecutive patients with uveal melanoma, Shields and associates found that 1.1% were children and teenagers younger than 20 years of age, of whom only 0.3% had ciliary body melanoma.

Patients with ciliary body melanoma usually are asymptomatic until the tumour impinges on the lens and causes visual distortion. Children with intraocular tumours generally have few visual symptoms and adapt to visual distortion without complaints. Leucocoria in childhood is the most frequent presenting sign of retinoblastoma, but it is generally not associated with uveal melanoma. We report an unusual case of a 9 year old child with a ciliary body melanoma who presented with leucocoria.

CASE REPORT

A 9 year old white girl was referred to Oncology Service at Wills Eye Hospital with a 1 month history of leucocoria and strabismus in her right eye (Fig 1A). She was otherwise healthy and her medical history was unremarkable.

Figure 1

Slit lamp photograph showing the inferonasal dark tumour, subluxing the cataractous lens, causing leucocoria (A). Gross pathology reveals …

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