Melanogenic neuroectodermal tumor of the retina (primary malignant melanoma of the retina).
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.
Freitag
S K
SK
Wills Eye Hospital, Jefferson Medical College of Thomas Jefferson University, Philadelphia, Pa 19107, USA.
Eagle
R C
RC
Shields
J A
JA
Duker
J S
JS
Font
R L
RL
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
D000951:Antigens, Neoplasm; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins