Pinealoblastoma with prominent retinoblastic differentiation: an unusual case in an adult.

We present an extremely rare case of pinealoblastoma with retinoblastic differentiation in a 32-year-old woman who presented with a history of intermittent headache of 2 years duration and diminution of vision for 2 months which eventually lead to total loss of vision. The fundus examination showed bilateral secondary optic atrophy. She did not have any previous history of retinoblastoma. The family history was non-contributory. Paraffin section of the tumor showed a primitive neuroectodermal tumor with numerous Flexner-Wintersteiner rosettes and the tumor cells were strongly positive for synaptophysin and negative for GFAP, S-100 protein and epithelial membrane antigen. This is the first case in the literature of a sporadic case of pinealoblastoma with prominent retinoblastic differentiation as evidenced histomorphologically by the presence of numerous Flexner-Wintersteiner rosettes in an adult female.