Sk. Freitag et al., MELANOGENIC NEUROECTODERMAL TUMOR OF THE RETINA (PRIMARY MALIGNANT-MELANOMA OF THE RETINA), Archives of ophthalmology, 115(12), 1997, pp. 1581-1584
A 35-month-old girl with leukocoria was clinically diagnosed with unil
ateral sporadic retinoblastoma. Macroscopic examination of her enuclea
ted eye disclosed a white retinal tumor that appeared to be a retinobl
astoma. Histopathologic examination, however, revealed that the tumor
was composed of poorly differentiated neuroblastic cells, larger spind
le-shaped cells, and anaplastic epithelioid cells, which is inconsiste
nt with retinoblastoma. Immunohistochemical testing disclosed that the
tumor cells were immunoreactive for melanoma-specific antigen HMB-45,
while electron microscopy showed premelanosomes in the tumor cells, b
oth of which are consistent with melanogenesis. To our knowledge, such
an ocular tumor has not been reported previously.