title: Melanogenic neuroectodermal tumor of the retina (primary malignant melanoma of the retina).
abstract: A 35-month-old girl with leukocoria was clinically diagnosed with unilateral sporadic retinoblastoma. Macroscopic examination of her enucleated eye disclosed a white retinal tumor that appeared to be a retinoblastoma. Histopathologic examination, however, revealed that the tumor was composed of poorly differentiated neuroblastic cells, larger spindle-shaped cells, and anaplastic epithelioid cells, which is inconsistent 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, both of which are consistent with melanogenesis. To our knowledge, such an ocular tumor has not been reported previously.
authors.affiliation: Wills Eye Hospital, Jefferson Medical College of Thomas Jefferson University, Philadelphia, Pa 19107, USA.
authors.forename: S K
authors.initials: SK
authors.lastname: Freitag
authors.forename: R C
authors.initials: RC
authors.lastname: Eagle
authors.forename: J A
authors.initials: JA
authors.lastname: Shields
authors.forename: J S
authors.initials: JS
authors.lastname: Duker
authors.forename: R L
authors.initials: RL
authors.lastname: Font
mesh_terms: D000951:Antigens, Neoplasm / Q000032:analysis; D002675:Child, Preschool; D003937:Diagnosis, Differential; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008545:Melanoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins / Q000032:analysis; D017600:Neuroectodermal Tumor, Melanotic / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D019572:Retinal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D012175:Retinoblastoma / Q000175:diagnosis
chemical_list: D000951:Antigens, Neoplasm; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins