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The eye is really an extension of the central nervous system, directly connected to the brain by the optic nerves (cranial nerve II). As such, examination of the eye affords the examiner a direct inspection of the central nervous system. The eye is surrounded by soft tissue and skin and many diseases affecting these tissues also affect the eye.

Inflammatory Pseudotumor of the Orbit
Ocular Melanoma
Retinitis Pigmentosa


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Clinicopathologic Correlations in 646 Consecutive Surgical Eye Specimens, 1990-2000

Anna S. Kitzmann,1 Amy L. Weaver, MS, Christine M. Lohse, Helmut Buettner, MD, and Diva R. Salomão, MD

Am J Clin Pathol 2003;119:594-601 Abstract quote

All surgical eye specimens examined in our pathology laboratory between January 1, 1990, and July 31, 2000 (N = 646), were classified by surgical procedure (enucleation, evisceration, or exenteration) and pathologic diagnosis.

Among 523 enucleated globes (81.0%), 252 (48.2%) contained tumors, of which 208 were intraocular malignant melanomas. Nonneoplastic causes for enucleation included glaucoma (67/523 [12.8%]), phthisis bulbi (61/523 [11.7%]), and recent trauma (59/523 [11.3%]). Sixty-seven specimens (10.4%) had been eviscerated.

Fifty-six specimens (8.7%) were obtained by exenteration performed to manage malignant tumors originating in or invading the orbit. Between 1990 and 2000, the percentage of procedures performed to remove neoplasms decreased from 65% to 38%, procedures performed to treat glaucoma increased from 4% to 29%, and those to treat phthisis bulbi increased from 8% to 13%.

The numbers of procedures remained relatively constant for trauma and for infectious and inflammatory diseases.



Failure to detect human papillomavirus DNA in malignant epithelial neoplasms of conjunctiva by polymerase chain reaction.

Eng HL, Lin TM, Chen SY, Wu SM, Chen WJ.

Department of Pathology, Kaohsiung Medical Center, Chang Gung University and Memorial Hospital, Taiwan, Republic of China.

Am J Clin Pathol 2002 Mar;117(3):429-36 Abstract quote

To elucidate the putative role of human papillomavirus (HPV) infection in the etiology of conjunctival tumors, 44 formalin-fixed, paraffin-embedded specimens of conjunctival tumors (24 patients with papillomas and 20 patients with dysplastic and/or malignant tumors) were screened for HPV infection using 4 different polymerase chain reactions (PCRs).

Of the 24 samples of papilloma, 14 (58%) displayed positive results by applying nested PCR using primer sets of HPV consensus L1 region. HPV type 6 or 11 was detected in 9 cases of papilloma by type-specific primer sets, but none of them were positive for HPV type 16 or 18. However, by using the highly sensitive PCR technique, we failed to demonstrate the HPV DNA of HPV types 6, 11, 16, and 18 in any of the 20 malignant epithelial tumors of conjunctiva.

We conclude that HPV-6 or HPV-11 is present in a substantial percentage of conjunctival papillomas, which is in accordance with findings of previously reported studies. In contrast, malignant conjunctival carcinomas are not associated with HPV infection; other pathogenic mechanisms, such as UV light, probably are more important in the cause of these malignant lesions.

Primary Glial Tumor of the Retina With Features of Myxopapillary Ependymoma.

Hegyi L, Peston D, Theodorou M, Moss J, Olver J, Roncaroli F.

From the Departments of *Histopathology and daggerOphthalmology, Charing Cross Hospital, Hammersmith Hospitals NHS Trust, and double daggerDepartment of Neuropathology, Imperial College, London, UK.

Am J Surg Pathol. 2005 Oct;29(10):1404-1410. Abstract quote  

We report a primary retinal tumor with features of myxopapillary ependymoma. The lesion occurred in a 33-year-old man with a long history of phthisis bulbi and a more recent history of pain to the right eye. Enucleated ocular globe revealed a lesion occupying most of the retinal surface.

Histologically, the retina was replaced by a tumor composed of spindle cells with fibrillary cytoplasm and round to ovoid nuclei forming fascicles, perivascular pseudorosettes, microcysts, and deposition of extracellular mucins. Calcifications, metaplastic bone, and lymphoplasmacytic inflammatory infiltrate were also seen. Tumor cells expressed GFAP and S-100 and to lesser extent carbonic anhydrase II. The immunoreaction for EMA showed diffuse granular positivity, decorated a few extracellular lumina, and highlighted intracytoplasmic lumina in a few cells.

Ultrastructurally, there was accumulation of extracellular material between cells and around capillaries, long interdigitating cytoplasmic processes, extracellular lumina packed with microvilli, a few junctions evident around lumina, and some ciliary basal bodies and ciliary basal rootlets. As control cases, we also investigated expression of EMA and carbonic anhydrase II in an ocular globe with retinal gliosis and three cases of myxopapillary ependymoma of the cauda equina.

The lesion described here represents the first example of retinal tumor with features of myxopapillary ependymoma. Pathologic features and particularly expression of carbonic anhydrase II suggest a derivation from intrinsic glial cells of retina otherwise known as Muller cells.
Massive gliosis of the retina: Report of a case investigated by immunohistochemistry and clonality assays.

Inayama Y, Hanashi M, Yazawa T, Mitsui H, Kimura A, Ito D.

Hum Pathol. 2005 Jun;36(6):702-5 Abstract quote.  

Summary We report a rare case of massive retinal gliosis that developed in a 32-year-old woman who had been born with bilateral microphthalmia. The patient had recently noticed left ophthalmos and underwent total resection of the affected eyeball.

Histologically, the vitreous body had been totally replaced by massively proliferated spindle cells, which had delicate fibrillary cytoplasm without nuclear atypia. Because the attenuated retinal pigment epithelium and intact sclera were preserved at the periphery of the tumor, the tumor was thought to be retinal in origin. Immunohistochemically, the spindle cells were strongly positive for glial fibrillary acidic protein and neuron-specific enolase and partly positive for S-100 protein. These findings led to a diagnosis of massive gliosis of the retina.

Clonality analysis of the tumor using a human androgen receptor assay revealed the polyclonal nature of the proliferating spindle cells. This is the first documentation of the polyclonality of this disease.

Bilateral ocular malformations in a newborn with normal karyotype: Histologic findings.

Pecorella I, Novacco V, Dadalt S, Ciardi A, Salvati G, Santillo C.

Department of Experimental Medicine and Pathology and the Institute of Opththalmology, University "La Sapienza," Rome; the Ophthalmologic Unit, Avezzano Hospital Avezzano; and the Ophthalmologic Unit, S. Carlo di Nancy Hospital, Rome, Italy.

Ann Diagn Pathol 2002 Oct;6(5):319-25 Abstract quote

Microphthalmos with cyst is a rare condition characterized by a small globe and an inferior uveoretinal coloboma. There is also a defect in the posterior aspect of the eye through which a cyst lined by neuroectodermically derived tissue protrudes into the orbit.

A case of isolated bilateral colobomatous and cystic microphthalmos is reported in an otherwise healthy child, showing no evidence of chromosomal abnormalities.

Microscopic findings in the enucleated eye consisted of iris and retinal dysgenesis, ectopia lentis, persistent anterior tunica vasculosa lentis and pupillary membrane, intrachoroidal smooth muscle, and optic nerve hypoplasia. In the orbital cyst, a thick membrane reminiscent of the retinal inner limiting membrane lay between the fibroadipose and vascularised outer wall and the inner neuroectodermal lining.

Vascular tumors in the orbit-capillary and cavernous hemangiomas.

Rosca TI, Pop MI, Curca M, Vladescu TG, Tihoan CS, Serban AT, Bontas EA, Gherghescu G.

Neurosurgery Unit, Clinical Emergency Hospital "St. Pantelimon," Bucharest, OP 22 Romania.

Ann Diagn Pathol. 2006 Feb;10(1):13-9. Abstract quote  

Vascular tumors in the orbit result from new formation of vessels, proliferation of tissue components of the vessel wall, and hyperplasia of cellular elements ordinarily concerned with the genesis of vascular tissue.

These vasculogenic lesions constitute the largest group of primary orbital tumors; we present the capillary hemangioma and the cavernous hemangioma.


Tumors and PseudoTumors of the Neurosensory Retina NUMBER OF CASES (AFIP registry from 1984-1989)
Retinoblastoma 188
Coats disease
Toxacara endophthalmitis
Persistent hyperplastic primary vitreous
Capillary hemangioma of von Hippel 4
Cavernous hemangioma 1
Astrocytoma 1
Astrocytic hamartoma (tuberous sclerosis) 1
Malignant lymphoma 14
Leukemia 3

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Last Updated February 8, 2006

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