- •Contents
- •General Introduction
- •Objectives
- •Organization
- •Topography
- •Disease Process
- •General Diagnosis
- •Differential Diagnosis
- •2 Wound Repair
- •General Aspects of Wound Repair
- •Healing in Specific Ocular Tissues
- •Cornea
- •Sclera
- •Limbus
- •Uvea
- •Lens
- •Retina
- •Vitreous
- •Eyelid, Orbit, and Lacrimal Tissues
- •Histologic Sequelae of Ocular Trauma
- •3 Specimen Handling
- •Communication
- •Orientation
- •Transillumination
- •Gross Dissection
- •Processing and Staining
- •Fixatives
- •Tissue Processing
- •Tissue Staining
- •4 Special Procedures
- •Immunohistochemistry
- •Flow Cytometry
- •Molecular Pathology
- •Diagnostic Electron Microscopy
- •Special Techniques
- •Fine-Needle Aspiration Biopsy
- •Frozen Section
- •5 Conjunctiva
- •Topography
- •Congenital Anomalies
- •Choristomas
- •Hamartomas
- •Inflammations
- •Papillary Versus Follicular Conjunctivitis
- •Granulomatous Conjunctivitis
- •Infectious Conjunctivitis
- •Noninfectious Conjunctivitis
- •Pyogenic Granuloma
- •Degenerations
- •Pinguecula and Pterygium
- •Amyloid Deposits
- •Epithelial Inclusion Cyst
- •Neoplasia
- •Squamous Lesions
- •Melanocytic Lesions
- •Lymphocytic Lesions
- •Glandular Lesions
- •Other Neoplasms
- •6 Cornea
- •Topography
- •Introduction to Corneal Pathology
- •Congenital Anomalies
- •Congenital Hereditary Endothelial Dystrophy
- •Posterior Polymorphous Dystrophy
- •Dermoid
- •Peters Anomaly
- •Inflammations
- •Infectious Keratitis
- •Noninfectious Keratitis
- •Degenerations and Dystrophies
- •Degenerations
- •Dystrophies
- •Neoplasia
- •7 Anterior Chamber and Trabecular Meshwork
- •Topography
- •Congenital Anomalies
- •Primary Congenital Glaucoma
- •Anterior Segment Dysgenesis
- •Degenerations
- •Iridocorneal Endothelial Syndrome
- •Secondary Glaucoma With Material in the Trabecular Meshwork
- •8 Sclera
- •Topography
- •Episclera
- •Stroma
- •Lamina Fusca
- •Congenital Anomalies
- •Choristoma
- •Nanophthalmos
- •Inflammations
- •Episcleritis
- •Scleritis
- •Degenerations
- •Senile Calcific Plaque
- •Scleral Staphyloma
- •Neoplasia
- •Fibrous Histiocytoma
- •Nodular Fasciitis
- •9 Lens
- •Topography
- •Capsule
- •Epithelium
- •Cortex and Nucleus
- •Zonular Fibers
- •Congenital Anomalies
- •Congenital Aphakia
- •Lens Coloboma
- •Anterior Lenticonus (Lentiglobus)
- •Posterior Lenticonus (Lentiglobus)
- •Inflammations
- •Phacoantigenic Uveitis
- •Phacolytic Glaucoma
- •Propionibacterium acnes Endophthalmitis
- •Degenerations
- •Cataract and Other Abnormalities
- •Neoplasia and Associations With Systemic Disorders
- •Pathology of Intraocular Lenses
- •10 Vitreous
- •Topography
- •Congenital Anomalies
- •Persistent Fetal Vasculature
- •Bergmeister Papilla
- •Mittendorf Dot
- •Prepapillary Vascular Loops
- •Vitreous Cysts
- •Inflammations
- •Degenerations
- •Syneresis and Aging
- •Posterior Vitreous Detachment
- •Rhegmatogenous Retinal Detachment and Proliferative Vitreoretinopathy
- •Macular Holes
- •Hemorrhage
- •Asteroid Hyalosis
- •Vitreous Amyloidosis
- •Neoplasia
- •Intraocular Lymphoma
- •11 Retina and Retinal Pigment Epithelium
- •Topography
- •Neurosensory Retina
- •Retinal Pigment Epithelium
- •Congenital Anomalies
- •Albinism
- •Myelinated Nerve Fibers
- •Vascular Anomalies
- •Congenital Hypertrophy of the RPE
- •Inflammations
- •Infectious
- •Noninfectious
- •Degenerations
- •Typical and Reticular Peripheral Cystoid Degeneration and Retinoschisis
- •Lattice Degeneration
- •Paving-Stone Degeneration
- •Ischemia
- •Specific Ischemic Retinal Disorders
- •Diabetic Retinopathy
- •Retinopathy of Prematurity
- •Age-Related Macular Degeneration
- •Polypoidal Choroidal Vasculopathy
- •Macular Dystrophies
- •Diffuse Photoreceptor Dystrophies
- •Neoplasia
- •Retinoblastoma
- •Retinocytoma
- •Medulloepithelioma
- •Fuchs Adenoma
- •Combined Hamartoma of the Retina and RPE
- •Adenomas and Adenocarcinomas of the RPE
- •12 Uveal Tract
- •Topography
- •Iris
- •Ciliary Body
- •Choroid
- •Congenital Anomalies
- •Aniridia
- •Coloboma
- •Inflammations
- •Infectious
- •Noninfectious
- •Degenerations
- •Rubeosis Iridis
- •Hyalinization of the Ciliary Body
- •Choroidal Neovascularization
- •Neoplasia
- •Iris
- •Choroid and Ciliary Body
- •Metastatic Tumors
- •Other Uveal Tumors
- •Trauma
- •13 Eyelids
- •Topography
- •Congenital Anomalies
- •Distichiasis
- •Phakomatous Choristoma
- •Dermoid Cyst
- •Inflammations
- •Infectious
- •Noninfectious
- •Degenerations
- •Xanthelasma
- •Amyloid
- •Cysts
- •Epidermoid and Dermoid Cysts
- •Ductal Cysts
- •Neoplasia
- •Epidermal Neoplasms
- •Dermal Neoplasms
- •Appendage Neoplasms
- •Melanocytic Neoplasms
- •14 Orbit
- •Topography
- •Bony Orbit and Soft Tissues
- •Congenital Anomalies
- •Dermoid and Other Epithelial Cysts
- •Inflammations
- •Noninfectious
- •Infectious
- •Degenerations
- •Amyloid
- •Neoplasia
- •Lacrimal Sac Neoplasia
- •Lacrimal Gland Neoplasia
- •Lymphoproliferative Lesions
- •Soft-Tissue Tumors
- •Vascular Tumors
- •Tumors With Fibrous Differentiation
- •Tumors With Muscle Differentiation
- •Nerve Sheath Tumors
- •Adipose Tumors
- •Bony Lesions of the Orbit
- •Metastatic Tumors
- •15 Optic Nerve
- •Topography
- •Congenital Anomalies
- •Colobomas
- •Inflammations
- •Infectious
- •Noninfectious
- •Degenerations
- •Optic Atrophy
- •Drusen
- •Neoplasia
- •Melanocytoma
- •Glioma
- •Meningioma
- •17 Melanocytic Tumors
- •Introduction
- •Iris Nevus
- •Nevus of the Ciliary Body or Choroid
- •Melanocytoma of the Iris, Ciliary Body, or Choroid
- •Iris Melanoma
- •Melanoma of the Ciliary Body or Choroid
- •Diagnostic Evaluation
- •Differential Diagnosis
- •Classification
- •Metastatic Evaluation
- •Treatment
- •Prognosis and Prognostic Factors
- •Pigmented Epithelial Tumors of the Uvea and Retina
- •Adenoma and Adenocarcinoma
- •Acquired Hyperplasia
- •Combined Hamartoma
- •18 Angiomatous Tumors
- •Hemangiomas
- •Choroidal Hemangiomas
- •Retinal Angiomas
- •Arteriovenous Malformation
- •19 Retinoblastoma
- •Genetic Counseling
- •Diagnostic Evaluation
- •Clinical Examination
- •Differential Diagnosis
- •Classification
- •Associated Conditions
- •Retinocytoma
- •Trilateral Retinoblastoma
- •Treatment
- •Enucleation
- •Chemotherapy
- •Photocoagulation and Hyperthermia
- •Cryotherapy
- •External-Beam Radiation Therapy
- •Plaque Radiotherapy (Brachytherapy)
- •Targeted Therapy
- •Spontaneous Regression
- •Prognosis
- •20 Ocular Involvement in Systemic Malignancies
- •Secondary Tumors of the Eye
- •Metastatic Carcinoma
- •Direct Intraocular Extension
- •Lymphomatous Tumors
- •Primary Intraocular Lymphoma
- •Uveal Lymphoid Infiltration
- •Ocular Manifestations of Leukemia
- •Basic Texts
- •Related Academy Materials
- •Requesting Continuing Medical Education Credit
Chévez-Barrios P. Frozen section diagnosis and indications in ophthalmic pathology. Arch Pathol Lab Med. 2005;129(12):1626– 1634.
Dermal Neoplasms
Capillary hemangiomas are common in the eyelids of children. They usually appear at or shortly after birth as a bright red lesion, grow over weeks to months, and involute by school age. Intervention is reserved for those lesions that affect vision because of ptosis or astigmatism, promoting amblyopia.
Figure 13-19 Squamous cell carcinoma. A, Clinical appearance. Note the focal loss of lashes and scaly appearance of the lower eyelid. B, Note the tumor cells (T) invading the dermis. C, Keratin (asterisk) is produced in this well-differentiated
squamous cell carcinoma. (Part A courtesy of Keith D. Carter, MD.)
The histopathologic appearance depends on the stage of evolution of the hemangioma. Early lesions may be very cellular, with solid nests of plump endothelial cells and correspondingly little vascular luminal formation. Established lesions typically show well-developed, flattened, endothelium-lined capillary channels in a lobular configuration (Fig 13-20). Involuting lesions demonstrate increased fibrosis and hyalinization of capillary walls with luminal occlusion.
Figure 13-20 Capillary hemangioma. A, Infant with multiple capillary hemangiomas. B, Note the small capillary-sized vessels and the proliferation of benign endothelial cells. (Part A courtesy of Sander Dubovy, MD.)
Appendage Neoplasms
Syringoma
Syringoma is a common benign lesion of the lower eyelid and typically manifests as multiple tiny papules. Syringomas result from a malformation of the eccrine sweat gland ducts. Histologically, syringomas consist of multiple, comma-shaped or round ductules lined with a double layer of epithelium and containing a central lumen, often with secretory material (Fig 13-21).
Sebaceous hyperplasia
Sebaceous hyperplasia is an uncommon benign lesion of the eyelid and face. Clinically, it appears as a small, yellow papule. Histologically, it is typically a single enlarged sebaceous gland with numerous glandular lobules attached to a single central duct (Fig 13-22).
Sebaceous adenoma
Sebaceous adenoma is a rare benign lesion of the eyelid that typically manifests as a yellow, circumscribed nodule. Histologically, it is composed of multiple sebaceous lobules that are irregularly shaped and incompletely differentiated (Fig 13-23). Muir-Torre syndrome should be considered when sebaceous adenoma is diagnosed (see Table 13-3).
Sebaceous carcinoma
A sebaceous carcinoma most commonly involves the upper eyelid of elderly persons. It may originate in the meibomian glands of the tarsus, the glands of Zeis in the skin of the eyelid, or the sebaceous glands of the caruncle. Clinical diagnosis is often missed or delayed because of this lesion’s propensity to mimic a chalazion or chronic blepharoconjunctivitis (Fig 13-24).
Figure 13-21 Histologically, syringoma is composed of small, epithelial-lined ductules that are round or comma-shaped
(arrows). (Courtesy of Nasreen A. Syed, MD.)
Figure 13-22 Sebaceous hyperplasia. Numerous sebaceous lobules (arrowheads) surround a hair follicle (arrow). (Courtesy of
Nasreen A. Syed, MD.)
Figure 13-23 Sebaceous adenoma. Sebaceous lobules demonstrate focal proliferations of basophilic (blue) sebocytes. This lesion is most commonly associated with Muir-Torre syndrome. (Courtesy of Nasreen A. Syed, MD.)
Histologically, well-differentiated sebaceous carcinomas are readily identified by the microvesicular foamy nature of the tumor cell cytoplasm (Fig 13-25A). Moderately differentiated tumors may exhibit some degree of sebaceous differentiation. Poorly differentiated tumors, however, may be difficult to distinguish from the other, more common epithelial malignancies. The demonstration of lipid within the cytoplasm of tumor cells by special stains, such as oil red O or Sudan black, is diagnostic, but it must be performed on tissue prior to processing and paraffin embedding.
When sebaceous carcinoma is suspected clinically, the pathologist should be alerted so that frozen section slides can be generated for lipid stains. Another feature, characteristic of but not pathognomonic for sebaceous cell carcinoma, is the dissemination of individual tumor cells and clusters of tumor cells within the epidermis or conjunctival epithelium, known as pagetoid spread (Fig 13-25B). Another pattern in the conjunctiva is that of complete replacement of conjunctival epithelium by tumor cells, or sebaceous carcinoma in situ (Fig 13-25C). A rare variant of sebaceous carcinoma involves only the epidermis and conjunctiva without demonstrable invasive tumor.
Figure 13-24 Sebaceous carcinoma. A, Note the eyelid erythema suggesting blepharitis. Note also the loss of eyelashes and the irregular eyelid thickening. B, This lesion mimics a chalazion of the lower eyelid. Focal lash loss is present.
courtesy of Roberta E. Gausas, MD.)
