[Skip to Content]
[Skip to Content Landing]
November 1996

Immunocytochemical Features of Retinoblastoma in an Adult

Author Affiliations

From the Department of Ophthalmology and Visual Sciences, University of Wisconsin Medical School, Madison (Drs Nork, de Venecia, Myers, and Vogel) and the Department of Ophthalmology, West Virginia University School of Medicine, Morgantown (Dr Millecchia).

Arch Ophthalmol. 1996;114(11):1402-1406. doi:10.1001/archopht.1996.01100140602013

Objective:  To describe the clinical course and immunocytochemical characteristics of an unusual intraocular tumor.

Methods:  Immunocytochemical analysis of the enucleated eye with an intraocular mass that markedly waxed and waned in size during 1 year of close observation of a 29-year-old woman.

Results:  Most of the tumor was composed of either dying or rapidly proliferating cells. One area located near the retina consisted mostly of well-differentiated cells in uniform sheets (bacillettes) with lacelike glial processes between the tumor cells. Almost all of the differentiated tumor cells were positive for S antigen. In particular, the dominant cell type stained positively for both antibodies known to be specific for those isoforms of S antigen found only in blue cones and rods but not in red or green cones. Only a few of these cells labeled positively with an anti—rhodopsin antibody.

Conclusions:  This is the first case of adult retinoblastoma to be confirmed immunocytochemically. The tumor was unusual because the differentiated regions contained bacillettes composed mostly of blue cones. It is possible that this and other adult retinoblastomas may arise from previously existing retinocytomas.

Pendergrass TW, Davis S.  Incidence of retinoblastoma in the United States . Arch Ophthalmol . 1980;98:1204-1210.Article
Shields CL, Shields JA, Shah P.  Retinoblastoma in older children . Ophthalmology . 1991;98:395-399.Article
Maghy C.  A case of bilateral glioma of the retina in a girl twenty years of age in which the second eye was excised after an interval of nearly eighteen years . Br J Ophthalmol . 1919;3:336-340.Article
Verhoeff FH.  Retinoblastoma: report of a case in a man aged forty-eight . Arch Ophthalmol . 1929;2:643-650.Article
Voznesenskaya AL.  Case of late development of glioma of the retina . Sovetskyi Vestnik Oftalmologii . 1933;2:202-204.
Oribe M, Malbran J.  Consideraciones anátomoclínicas sobre los gliomas de la retina (su conocimiento actual) . Archiv Oftalmol Buenos Aires . 1938;13:319-353.
McCrea WBE.  Glioma of the retina: review of 12 cases . Br J Ophthalmol . 1943;27:259-273.Article
Rasmussen K.  Retinoblastoma in a man aged forty-eight years . Acta Ophthalmol . 1943;21:210-213.Article
O'Day K.  Two intraocular tumours of unusual interest . Trans Ophthalmol Soc Aust . 1947;7:81-86.
Rychener RO.  Retinoblastoma in the adult . Trans Am Ophthalmol Soc . 1948;46:318-326.
Arseni C, Opresco I.  Quelques particularités anatomo-cliniques du rétinoblastome . Ann Anat Patholog . 1959;4:129-135.
Mehra KS, Hamid S.  Retinoblastoma in an adult . Am J Ophthalmol . 1961;52:405-406.
Finlay JR, Byron H.  Retinoblastoma in the adult: review of literature and report of case associated with a benign melanoma . In: International Congress of Ophthalmology, 1962 . New Delhi, India: Excerpta Medica Foundation; 1962:1168-1178.
Makley Jr TA.  Retinoblastoma in a 52-year-old man . Arch Ophthalmol . 1963;69:325-327.Article
Ohara H, Akatuka S, Akutu S, Matui N.  A case of retinoblastoma in an aged woman . J Clin Ophthalmol (Tokyo) . 1963;17:951-953.
Lasch H.  Ein Retinoblastomrezidiv im Erwachsenenalter . Klin Monatsbl Augenheilkd . 1964;144:268-272.
Perz M, Majewski C.  Przypadek siatkówczaka (retinoblastoma) u dorostego mȩźczyzny . Klin Oczna . 1964;34:261-264.
Toselli C, Costa V.  Rilievi ultrastrutturali sul retinoblastoma nell'adulto [Ultra-structural studies of retinoblastoma in the adult] . Ann Ottalmol Clin Oculist . 1968;94:435-447.
Kremlicka L, Roubková H.  Retinoblastom im reifen Alter . Klin Monatsbl Augenheilkd . 1975;167:119-123.
Berkeley JS, Kalita BC.  Retinoblastoma in an adult . Lancet . 1977;2:508-509.Article
Takahashi T, Tamura S, Inoue M, Isayama Y, Sashikata T.  Retinoblastoma in a 26-year-old adult . Ophthalmology . 1983;90:179-183.Article
Neronova-Kotova LA.  Sluchai retinoblastomy u vzroslogo [Case of retinoblastoma in an adult] . Vest Oftalmol . 1986;102:75.
Shuangshoti S, Chaiwun B, Kasantikul V.  A study of 39 retinoblastomas with particular reference to morphology, cellular differentiation and tumour origin . Histopathology . 1989;15:113-124.Article
Balmer A, Munier F, Gailloud C.  Retinoma: case studies . Ophthalmic Paediatr Genet . 1991;12:131-137.Article
Jakobiec FA, Brodie SE, Haik B, Iwamoto T.  Giant cell astrocytoma of the retina: a tumor of possible Mueller cell origin . Ophthalmology . 1983;90:1565-1576.Article
Molnar ML, Stefansson K, Marton LS, Tripathi RC, Molnar GK.  Distribution of S-100 protein and glial fibrillary acidic protein in normal and gliotic human retina . Exp Eye Res . 1984;38:27-34.Article
Eisenfeld AJ, Bunt-Milam AH, Sarthy PV.  Muller cell expression of glial fibrillary acidic protein after genetic and experimental photoreceptor degeneration in the rat retina . Invest Ophthalmol Vis Sci . 1984;25:1321-1328.
Nork TM, Ghobrial MW, Peyman GA, Tso MOM.  Massive retinal gliosis: a reactive proliferation of Müller cells . Arch Ophthalmol . 1986;104:1383-1389.Article
Nork TM, Wallow IHL, Sramek SJ, Anderson G.  Müller's cell involvement in proliferative diabetic retinopathy . Arch Ophthalmol . 1987;105:1424-1429.Article
Bjorklund H, Bignami A, Dahl D.  Immunohistochemical demonstration of glial fibrillary acidic protein in normal rat Müller glia and retinal astrocytes . Neurosci Lett . 1985;54:363-368.Article
Hall PA, Levison DA, Woods AL, et al.  Proliferating cell nuclear antigen (PCNA) immunolocalization in paraffin sections: an index of cell proliferation with evidence of deregulated expression in some neoplasms . J Pathol . 1990;162:285-294.Article
Nork TM, Mangini NJ, Millecchia LL.  Rods and cones contain antigenically distinctive S-antigens . Invest Ophthalmol Vis Sci . 1993;34:2918-2925.
Adamus G, Arendt A, Zam ZS, McDowell JH, Hargrave PA.  Use of peptides to select for anti-rhodopsin antibodies with desired amino acid sequence specificities . Pept Res . 1988;1:42-47.
Büchi ER, Bernauer W, Daicker B.  Cell death and disposal in retinoblastoma: an electron microscopic  study. Graef Arch Clin Exp Ophthalmol . 1994;232:635-645.Article
Nork TM, Schwartz TL, Doshi HM, Millecchia LL.  Retinoblastoma: cell of origin . Arch Ophthalmol . 1995;113:791-802.Article
Boniuk M, Bishop DW.  Oligodendroglioma of the retina . Surv Ophthalmol . 1969;13:284-289.
He W, Hashimoto H, Tsuneyoshi M, Enjoji M, Inomata H.  A reassessment of histologic classification and an immunohistochemical study of 88 retinoblastomas: a special reference to the advent of bipolar-like cells . Cancer . 1992;70:2901-2908.Article
Margo C, Hidayat A, Kopelman J, Zimmerman LE.  Retinocytoma: a benign variant of retinoblastoma . Arch Ophthalmol . 1983;101:1519-1531.Article
Gallie BL, Ellsworth RM, Abramson DH, Phillips RA.  Retinoma: spontaneous regression of retinoblastoma or benign manifestation of the mutation? Br J Cancer . 1982;45:513-521.Article
Gallie BL, Phillips RA, Ellsworth RM, Abramson DH.  Significance of retinoma and phthisis bulbi for retinoblastoma . Ophthalmology . 1982;89:1393-1399.Article
Onadim Z, Hogg A, Baird PN, Cowell JK.  Oncogenic point mutations in exon 20 of the RB1 gene in families showing incomplete penetrance and mild expression of the retinoblastoma phenotype . Proc Natl Acad Sci U S A . 1992;89:6177-6181.Article
Dryja TP, Rapaport J, McGee TL, Nork TM, Schwartz TL.  Molecular etiology of low-penetrance retinoblastoma in two pedigrees . Am J Hum Genet . 1993;52:1122-1128.
Kratzke RA, Otterson GA, Hogg A, et al.  Partial inactivation of the RB product in a family with incomplete penetrance of familial retinoblastoma and benign retinal tumors . Oncogene 1994;9:1321-1326.
Eagle R, Jr, Shields JA, Donoso L, Milner RS.  Malignant transformation of spontaneously regressed retinoblastoma, retinoma/retinocytoma variant . Ophthalmology . 1989;96:1389-1395.Article
Shields CL, Shields JA, Baez KA, Cater J, De Potter PV.  Choroidal invasion of retinoblastoma: metastatic potential and clinical risk factors . Br J Ophthalmol . 1993;77:544-548.Article