Concurrent pineoblastoma and unilateral retinoblastoma: a forme fruste of trilateral retinoblastoma?
A 10-month-old boy who presented with strabismus and symptoms of intracranial hypertension was found to have a pineoblastoma and a unilateral ocular retinoblastoma. Despite enucleation of the eye, subtotal removal of the pineoblastoma, and craniospinal axis irradiation, the patient died 6 months later from disseminated intracranial neoplasm. As there was no clinical evidence of bilateral retinoblastoma, this case may represent a forme fruste of the trilateral retinoblastoma complex. The clinicopathological features of this unusual syndrome are reviewed.
D001932:Brain Neoplasms / Q000473:pathology / Q000601:surgery*; D002557:Cerebrospinal Fluid Shunts; D003131:Combined Modality Therapy; D005134:Eye Neoplasms / Q000473:pathology / Q000601:surgery*; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery; D007223:Infant; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology / Q000601:surgery*; D010870:Pineal Gland / Q000473:pathology; D010871:Pinealoma / Q000473:pathology / Q000601:surgery*; D012160:Retina / Q000473:pathology; D012175:Retinoblastoma / Q000473:pathology / Q000601:surgery*; D014057:Tomography, X-Ray Computed