Ocular Cytopathology

An atlas that features the cytologic findings of the normal features and diseases of the eye.

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Sunday, October 02, 2005


In this chapter, malignant neoplasms of the eye are illustrated. Iris and ciliary body melanomas were presented in Chapter 5. Orbital and periorbital tumors are discussed in Chapter 10.
Only a few malignant tumors originate in the eye. Interpretation of aspirates from these tumors is not difficult if sampling is adequate and one is familiar with their specific criteria. Some primary ocular tumors have different criteria for diagnosis than analogous systemic neoplasms (e.g., melanoma). Diagnosis of other tumors is complicated by inherent difficulty in obtaining adequate material (e.g., lymphoma).


Intraocular lymphomas are rare and usually associated with involvement of the central nervous system. [1, 2, 3, 4, 5] Patients frequently have blurred vision without pain and characteristic yellow lesions under the retinal pigment epithelium (Figure 9-1). [6] However, ocular involvement may be confined to the vitreous cavity. When vitreous cells are present, the diagnosis may be made by vitreous aspirate or vitrectomy. Cytospin preparation or direct smears of vitreous reveal atypical lymphoid cells with prominent nucleoli (Figure 9-2). Most intraocular lymphomas contain large cells that have B-cell immunophenotypic markers. Because of the scant material obtained, it is often very difficult to make an unequivocal diagnosis of malignancy. In addition, hyaluronic acid in the vitreous may nonspecifically bind antibody making immunphenotypic markers difficult to interpret. Some authors suggest that morphologic analysis is more accurate than immunophenotypic analysis (B-and T-cell markers) in this setting. [7, 8] Hyaluronidase-treated vitreous samples may improve the accuracy of immunphenotypic analysis by flow cytometry. [9]


Retinoblastoma manifests early in childhood as leukocoria. [10, 11] In the genetic form it is inherited in an autosomal dominant fashion, but at the molecular level it behaves as an autosomal recessive defect. [12, 13, 14, 15, 16] Retinoblastoma is associated with the homozygous deletion or alternation of a well-characterized gene in the chromosome region 13q14. [17, 18, 19, 20]
Retinoblastoma appears as a white mass arising from the retina (Figures 9-3 and 9-4). It is a small-cell tumor and is characterized by areas of necrosis, calcification, Flexner-Wintersteiner rosettes, Homer Wright rosettes, and, rarely, fleurettes (Figure 9-5). [21- 23, 24]Intraocular and orbital fine needle aspirations of retinoblastoma have been described. [25] However, it is generally recommended that fine needle aspiration and other procedures to sample retinoblastoma be avoided because seeding has been documented. [26, 27] This is a controversial subject. Cytologic preparations reveal a small cell tumor with necrosis and streaming of deoxyribonucleic acid (DNA) (Figure 9-6). [28] Rosettes have been reported, but they are rarely identified. [29]


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22. Tso MOM, Fine BS, Zimmerman LE. The Flexner-Wintersteiner rosettes in retinoblastoma. Arch Pathol 1969; 88:664-671.
23. Rodrigues MM, et al. Ophthalmology. 1987 Apr;94(4):378-87.
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26. Rodriguez A., Mod Probl Ophthalmol. 1977;18:142-8.
27. Karcioglu ZA, et al. Tumor seeding in ocular fine needle aspiration biopsy Ophthalmology. 1985 Dec;92(12):1763-7.
28. Rosenthal DL, Mandell DB, Glasgow BJ. Eye. In: Bibblo M, ed. Comprehensive cytology. Philadelphia: W.B. Saunders, 1991;484-501.
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