TUMORS OF THE EYE AND OCULAR ADNEXA

by

IAN W. MCLEAN, M.D.
Chairman, Department of Ophthalmic Pathology
Armed Forces Institute of Pathology
Washington, D.C.

MIGUEL N. BURNIER, M.D., Ph.D.
Professor of Ophthalmology and Pathology
Director, Eye Pathology Laboratory, and
Chairman, Department of Ophthalmology
McGill University
Montreal, Canada

LORENZ E. ZIMMERMAN, M.D.
Chairman Emeritus, Department of Ophthalmic Pathology
Armed Forces Institute of Pathology
Professor of Pathology and Ophthalmology, Georgetown University
Consultant in Ophthalmic Pathology
Washington Hospital Center
Washington, D.C.

FREDERICK A. JAKOBIEC, M.D.
Professor of Ophthalmology and Pathology
Chairman, Department of Ophthalmology,
Harvard Medical School
Chief of Ophthalmology,
Massachusetts Eye and Ear Infirmary
Boston, Massachusetts


Table of Contents

1 Introduction
2 Tumors of the Eyelid
 2.1 Anatomy and Histology
 2.2 Classification and Frequency
 2.3 Tumors of the Epidermis
  2.3.1 Squamous Cell Papilloma (Fibroepithelial Papilloma)
  2.3.2 Seborrheic Keratosis (Basal Cell Papilloma, Seborrheic Wart,
        Senile Verruca)
  2.3.3 Inverted Follicular Keratosis (Irritated Seborrheic Keratosis)
  2.3.4 Pseudocarcinomatous (Pseudoepitheliomatous) Hyperplasia
  2.3.5 Keratoacanthoma
  2.3.6 Actinic Keratosis (Solar Keratosis, Senile Keratosis)
  2.3.7 Bowen Disease
  2.3.8 Squamous Cell Carcinoma
  2.3.9 Basal Cell Carcinoma
 2.4 Benign Tumors of Eccrine and Apocrine Gland Origin
  2.4.1 Hydrocystoma
  2.4.2 Syringoma
  2.4.3 Eccrine Acrospiroma (Clear Cell Hidradenoma, Clear Cell
        Myoepithelioma, Eccrine Poroma, Porosyringoma)
  2.4.4 Pleomorphic Adenoma (Mixed Tumor of the Sweat Glands, Chondroid
        Syringoma)
 2.5 Malignant Tumors of Eccrine Sweat Gland Origin (Eccrine
     Adenocarcinoma)
 2.6 Apocrine Adenocarcinoma
 2.7 Sebaceous Gland Tumors
  2.7.1 Benign Tumors of Sebaceous Origin
  2.7.2 Sebaceous Carcinoma (Adenocarcinoma of Sebaceous Gland, Meibomian
        Gland Carcinoma, Zeis Gland Carcinoma)
 2.8 Tumors of Hair Follicle Origin
  2.8.1 Trichoepithelioma (Epithelioma Adenoides Cysticum, Multiple
        Benign Cystic Epithelioma, Brooke Tumor)
  2.8.2 Trichofolliculoma
  2.8.3 Trichilemmoma
  2.8.4 Pilomatrixoma (Calcifying Epithelioma of Malherbe)
 2.9 Adnexal Carcinoma
 2.10 Tumors of Melanocytes
 2.11 Tumors of Hematopoietic and Soft Tissues
  2.11.1 Hemangiomas
   2.11.1.1 Capillary Hemangioma (Juvenile Hemangioma, Strawberry Nevus,
            Nevus Vasculosus, Benign Hemangioendothelioma of Childhood)
   2.11.1.2 Nevus Flammeus (Port Wine Stain)
   2.11.1.3 Cavernous Hemangioma
 2.12 Miscellaneous Tumors
  2.12.1 Phakomatous Choristoma
  2.12.2 Merkel Cell Tumor (Trabecular Carcinoma of the Skin, Cutaneous
         APUDoma, Small Cell Neuroepithelial Tumor, Primary Small Cell
         Cutaneous Carcinoma,Neuroendocrine or Merkel Cell Carcinoma)
  2.12.3 Carcinoma Metastatic to the Eyelid
3 Tumors of the Conjunctiva
 3.1 Anatomy and Histology
  3.1.1 Gross Anatomic Features
  3.1.2 Microscopic Features
 3.2 Classification and Frequency
 3.3 Benign Tumors of Surface Epithelium
  3.3.1 Squamous Cell Papilloma
  3.3.2 Keratotic Plaque
  3.3.3 Pseudocarcinomatous Hyperplasia (Pseudoepitheliomatous
        Hyperplasia)
  3.3.4 Hereditary Benign Intraepithelial Dyskeratosis
 3.4 Intraepithelial Neoplasia of Surface Epithelium and Related Lesions
  3.4.1 Pinguecula, Pterygium, and Actinic Keratosis (Solar Keratosis)
  3.4.2 Dysplasia
  3.4.3 Carcinoma in Situ
 3.5 Malignant Tumors of Surface Epithelium
  3.5.1 Squamous Cell Carcinoma
  3.5.2 Mucoepidermoid Carcinoma
 3.6 Adnexal Tumors
  3.6.1 Oncocytoma (Oncocytic Adenoma, Oxyphilic Adenoma)
 3.7 Tumors of the Melanocytic System
  3.7.1 Nevi
  3.7.2 Melanosis
   3.7.2.1 Epithelial Congenital Melanosis
   3.7.2.2 Subepithelial Congenital Melanosis
   3.7.2.3 Secondary Melanosis
   3.7.2.4 Primary Acquired Melanosis (Atypical Melanocytic Hyperplasia,
           Malignant Melanoma in Situ, Benign Acquired Melanosis,
           Precancerous Melanosis)
 3.8 Malignant Melanoma
 3.9 Soft Tissue, Lymphoid, Hematopoietic, and Histiocytic Tumors
  3.9.1 Kaposi Sarcoma
 3.10 Choristomas
  3.10.1 Limbal Dermoid
  3.10.2 Dermolipoma
4 Tumors of the Retina
 4.1 Anatomy and Histology
  4.1.1 Retinal Pigment Epithelium
  4.1.2 Sensory (Neural) Retina
 4.2 Classification and Frequency
 4.3 Retinoblastoma, Retinocytoma, and Pseudoretinoblastoma
  4.3.1 Toxocara Canis Endophthalmitis
  4.3.2 Persistent Hyperplastic Primary Vitreous
  4.3.3 Coats Disease
 4.4 Glial Tumors and Tumor-Like Conditions
  4.4.1 Astrocytoma
  4.4.2 Massive Gliosis
 4.5 Vascular Tumors and Tumor-Like Conditions
  4.5.1 Angiomatosis Retinae (Hemangioblastoma, Capillary Hemangioma,
        von Hippel-Lindau Syndrome)
  4.5.2 Cavernous Hemangioma
 4.6 Tumors of Hematopoietic and Lymphoid Tissues
  4.6.1 Malignant Lymphoma
  4.6.2 Leukemia
 4.7 Neuroepithelial Tumors
  4.7.1 Glioneuroma
  4.7.2 Medulloepithelioma (Diktyoma, Teratoneuroma)
  4.7.3 Acquired Neuroepithelial Tumors of the Ciliary Body
  4.7.4 Fuchs Adenoma (Hyperplasia of the Nonpigmented Ciliary
        Epithelium, Pseudoadenomatous Hyperplasia, Coronal Adenoma)
  4.7.5 Adenomas of the Nonpigmented Ciliary Epithelium
  4.7.6 Carcinomas of the Nonpigmented Ciliary Epithelium
  4.7.7 Adenomas and Carcinomas of the Pigmented Ciliary Epithelium
  4.7.8 Adenomas and Carcinomas of the Retinal Pigment Epithelium
 4.8 Metastatic Neoplasms
5 Tumors of the Uveal Tract
 5.1 Anatomy and Histology
  5.1.1 The Iris
  5.1.2 The Ciliary Body
  5.1.3 The Choroid
 5.2 Classification and Frequency
 5.3 Melanocytic Nevi
 5.4 Malignant Melanoma of the Uveal Tract
 5.5 Hemangioma
 5.6 Choroidal Osteoma
 5.7 Leiomyoma and Mesectodermal Leiomyoma
 5.8 Leukemia
 5.9 Juvenile Xanthogranuloma
 5.10 Well-Differentiated Small Lymphocytic or Lymphoplasmacytic Lymphoma
      and Secondary Lymphomas of the Uvea
 5.11 Metastatic Tumors
6 Tumors of the Lacrimal Gland and Sac
 6.1 Anatomy and Histology
 6.2 Classification and Frequency
 6.3 Pleomorphic Adenoma (Benign Mixed Tumor)
 6.4 Pleomorphic Carcinoma (Malignant Mixed Tumor)
 6.5 Adenoid Cystic Carcinoma
 6.6 Adenocarcinoma Arising De Novo
 6.7 Tumors of the Lacrimal Sac
  6.7.1 Epithelial Tumors (Papillomas and Carcinomas)
7 Tumors of the Orbit
 7.1 Anatomy and Histology
 7.2 Classification and Frequency
 7.3 Capillary Hemangioma
 7.4 Cavernous Hemangioma
 7.5 Lymphangioma
 7.6 Hemangiopericytoma
 7.7 Fibrous Histiocytoma (Fibroxanthoma)
 7.8 Fibromatosis (Myofibromatosis, Juvenile Fibromatosis)
 7.9 Fibrosarcoma (Juvenile Fibrosarcoma)
 7.10 Leiomyoma
 7.11 Leiomyosarcoma
 7.12 Rhabdomyosarcoma
 7.13 Lipoma
 7.14 Liposarcoma
 7.15 Neurofibromas
  7.15.1 Plexiform Neurofibroma
  7.15.2 Diffuse Neurofibroma
  7.15.3 Isolated Neurofibroma
 7.16 Schwannoma (Neurilemoma)
 7.17 Malignant Peripheral Nerve Sheath Tumors
 7.18 Lymphoid Tumors and Inflammation
  7.18.1 Inflammatory Pseudotumors
  7.18.2 Graves Orbitopathy
  7.18.3 Idiopathic Orbital Inflammation (Pseudotumors)
  7.18.4 Lymphoid Tumors
   7.18.4.1 Lymphoid Hyperplasia (Benign Lymphoid Tumor)
   7.18.4.2 Malignant Lymphomas
   7.18.4.3 Follicular Lymphomas
   7.18.4.4 Well-Differentiated and Intermediately Differentiated Diffuse
            Lymphomas
   7.18.4.5 Poorly Differentiated Diffuse Lymphomas
 7.19 Plasma Cell Tumors
 7.20 Leukemia (Granulocytic Sarcoma)
 7.21 Histiocytic Disorders
  7.21.1 Unifocal and Multifocal Eosinophilic Granuloma (Langerhans Cell
         Histiocytosis)
  7.21.2 Sinus Histiocytosis with Massive Lymphadenopathy
 7.22 Dermoid Cyst
 7.23 Teratoma
 7.24 Congenital Melanosis, Cellular Blue Nevus, and Primary Malignant
      Melanoma
 7.25 Fibrous Dysplasia
 7.26 Ossifying Fibroma (Juvenile Ossifying Fibroma, Psammomatoid
      Ossifying Fibroma)
 7.27 Osteogenic Sarcoma
 7.28 Secondary Tumors of the Orbit
 7.29 Metastatic Tumors
8 Tumors of the Optic Nerve and Optic Nerve Head
 8.1 Anatomy and Histology
 8.2 Classification and Frequency
 8.3 Melanocytoma (Magnocellular Nevus)
 8.4 Malignant Melanoma
 8.5 Juvenile Pilocytic Astrocytoma
 8.6 Malignant Astrocytoma of Optic Nerve and Chiasm
 8.7 Meningioma
 8.8 Secondary and Metastatic Optic Nerve Tumors
9 Index

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