Background
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.
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CLINICAL VARIANTS CHARACTERIZATION GLIAL TUMOR WITH FEATURES OF MYXOPAPILLARY EPENDYMOMA
- 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
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.MICROPHTHALMOS WITH CYSTIC COLOBOMA 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 OF THE ORBIT
- 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.
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Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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Last Updated February 8, 2006
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