2 Eyelid, Conjunctival, and Orbital Tumors AN ATLAS AND TEXTBOOK THIRD EDITION Jerry A. Shields, MD Director, Ocular Oncology Service Wills Eye Hospital Professor of Ophthalmology Thomas Jefferson University Philadelphia, Pennsylvania, USA Carol L. Shields, MD Co-Director, Ocular Oncology Service Wills Eye Hospital Professor of Ophthalmology Thomas Jefferson University Philadelphia, Pennsylvania, USA 3 Acquisitions Editor: Kel McGowan Senior Product Development Editor: Emilie Moyer Marketing Manager: Stephanie Kindlick Senior Production Project Manager: Alicia Jackson Design Coordinator: Stephen Druding Manufacturing Coordinator: Beth Welsh Prepress Vendor: Aptara, Inc. Third edition Copyright © 2016 Wolters Kluwer. Copyright © 2007 Wolters Kluwer Health | Lippincott Williams & Wilkins. Copyright © 1999 Lippincott Williams & Wilkins, a Wolters Kluwer business. All rights reserved. This book is protected by copyright. No part of this book may be reproduced or transmitted in any form or by any means, including as photocopies or scanned-in or other electronic copies, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright. To request permission, please contact Wolters Kluwer at Two Commerce Square, 2001 Market Street, Philadelphia, PA 19103, via email at [email protected], or via our website at lww.com (products and services). 9 8 7 6 5 4 3 2 1 Printed in China Library of Congress Cataloging-in-Publication Data Shields, Jerry A., author. Eyelid, conjunctival, and orbital tumors : an atlas and textbook / Jerry A. Shields, Carol L. Shields. – Third edition. p. ; cm. Includes bibliographical references and index. ISBN 978-1-4963-2148-0 (alk. paper) I. Shields, Carol L., author. II. Title. [DNLM: 1. Eyelid Neoplasms–Atlases. 2. Conjunctival Neoplasms–Atlases. 3. Orbital Neoplasms–Atlases. WW 17] RC280.E9 616.99’484–dc23 2015021024 This work is provided “as is,” and the publisher disclaims any and all warranties, express or implied, including any warranties as to accuracy, comprehensiveness, or currency of the content of this work. This work is no substitute for individual patient assessment based upon healthcare professionals’ examination of each patient and consideration of, among other things, age, weight, gender, current or prior medical conditions, medication history, laboratory data and other factors unique to the patient. The publisher does not provide medical advice or guidance and this work is merely a reference tool. Healthcare professionals, and not the publisher, are solely responsible for the use of this work including all medical judgments and for any resulting diagnosis and treatments. Given continuous, rapid advances in medical science and health information, independent professional 4 verification of medical diagnoses, indications, appropriate pharmaceutical selections and dosages, and treatment options should be made and healthcare professionals should consult a variety of sources. When prescribing medication, healthcare professionals are advised to consult the product information sheet (the manufacturer’s package insert) accompanying each drug to verify, among other things, conditions of use, warnings and side effects and identify any changes in dosage schedule or contraindications, particularly if the medication to be administered is new, infrequently used or has a narrow therapeutic range. To the maximum extent permitted under applicable law, no responsibility is assumed by the publisher for any injury and/or damage to persons or property, as a matter of products liability, negligence law or otherwise, or from any reference to or use by any person of this work. LWW.com 5 This book is dedicated to our seven children, Jerry, Patrick, Bill, Maggie Mae, John, Nellie, and Mary Rose. They are now in their teens and twenties, and still remain most precious to us. We wish them satisfaction and success in their home life and careers and hope that they will flourish as they chase their dreams. 6 FOREWORD 1 Since Jerry Shields established the Ocular Oncology Service at Wills Eye Hospital in 1974, it has grown into one of the largest and best ocular oncology departments in the world. The ocular oncology team at Wills sees dozens of new patients every week, and performs surgery to treat tumors several days a week. The Service is now headed by Jerry and his wife Carol Shields, two giants in the field. Jerry and Carol and their team have published hundreds of papers in the peer-reviewed ophthalmic literature and lectured extensively throughout the United States and around the world on the diagnosis and management of ocular tumors. I can think of no better doctors to write textbooks dealing with tumors in and around the eye. The first two editions of the Eyelid, Conjunctival, and Orbital Tumors Atlas and Textbook were superb! This third edition has been updated to include more diagnoses, additional photographs, and cutting edge medical and surgical treatments. The book is divided into three parts which are subdivided into 41 chapters and contains over 2,600 high-quality images. Part 1 deals with tumors of the eyelid. It is composed of 15 chapters on all varieties of eyelid cancers including tumors of the epidermis such as basal cell and squamous cell carcinomas, sebaceous gland tumors, melanocytic tumors, vascular tumors, and numerous conditions simulating neoplasms. The last chapter in this part is on the surgical management of eyelid tumors. Part 2 deals with tumors of the conjunctiva including choristomas such as dermoids, intraepithelial neoplasia and invasive squamous cell carcinoma, vascular tumors, lymphoid tumors, and numerous conditions simulating conjunctival neoplasms. The last chapter in this part is on the surgical management of conjunctival tumors. Part 3 deals with tumors of the orbit including cystic lesions, vascular and hemorrhagic lesions, peripheral and optic nerve tumors, myogenic tumors such as rhabdomyosarcoma, lacrimal gland tumors, lymphoid tumors and leukemias, and numerous inflammatory conditions simulating orbital neoplasms. The last chapter in this part is on the surgical management of orbital tumors. Each chapter includes discussion of many different tumors or mimicking conditions. For each diagnostic entity, there are general considerations, clinical features, differential diagnosis, pathology, and management. This is followed by numerous superb photos of the clinical entity, imaging studies, pathology, and surgical management. Drs. Jerry and Carol Shields should be congratulated on improving on their already wonderful textbook with this third edition. As clinicians seeing patients with possible ocular and adnexal tumors, we should be extremely thankful to them for putting together such an impressive atlas and textbook! Christopher J. Rapuano, MD Chief of the Cornea Service, Co-Chief of the Refractive Surgery Department, Wills Eye Hospital Professor of Ophthalmology, Sidney Kimmel Medical College at Thomas Jefferson University Philadelphia, PA 7 FOREWORD 2 This Third Edition of the 2-volume set on ophthalmic tumors, the second of which is entitled “Eyelid, Conjunctival and Orbital Tumors: An Atlas and Textbook,” has been kept to a reasonable length of somewhat more than 800 pages yet embraces many valuable improvements. As an individual with a longstanding interest in orbital and adnexal tumors, I can say without equivocation or reservation that the second volume of the set devoted to the adnexa has no match and should be in every ophthalmologist’s and trainee’s (along with the first volume) collection of ophthalmic treatises. The accessibility of the knowledge that this volume contains has been facilitated by a highly logical unfolding of topics in the Table of Contents and an excellent Index. We are well into the era of internet publishing that threatens the very survival of books in both medical and non-medical fields. Today’s readers expect to obtain their knowledge in dollops and tweets which means they eschew more considered, in- depth treatments of topics found in textbooks. This is unfortunate. Familiarity with a benchmark source of reasonable length permits one to amble through it so that it becomes like an old friend or companion. While it is not possible to retain everything read in a major textbook, intimacy with it eases a return to it for easily finding the desired information. Even if books are fast assuming the status of an endangered species, there will always be a role and need for a select few that have passed the test of time. Literary critics refer to such intellectual artifacts that have unarguably transformed into classics as “canonical”—signifying their central and seminal significance among books of their genre. Other examples besides the present enterprise that qualify for this sobriquet are Miller and Newman’s revision of the 3-volume Walsh and Hoyt’s Clinical Neuro-Ophthalmology (Lippincott Williams & Wilkins, 2004), Spencer’s 4- volume Ophthalmic Pathology: An Atlas and Text (WB Saunders, 1995), and Miller and Albert’s 4-volume Albert and Jakobiec’s Principles and Practice of Ophthalmology (WB Saunders, 2007). Each section of this volume contains a thoroughly revised, scholarly and comprehensive introductory overview that covers in a succinct and practical fashion what the clinician needs to know about the diagnosis and management of tumors of the eyelids, conjunctiva, and orbit. More than 4,000 carefully chosen and beautiful color illustrations of the relevant clinical and histopathologic features, with a complement of black and white imaging studies, reinforce the points made in the text. The bibliographies for each topic are judiciously selected and up to date. Many new entities have been described since the last edition. Two that appeal to my pedantic side are neurothekeoma (occurring preferentially in the eyelids and exceptionally in the conjunctiva and orbit) and phacomatosis pigmentovascularis of the cesioflammea type. The latter condition combines a nevus flammeus with ipsilateral ocular melanosis, melanocytosis, or melanocytic tumors. The nevus flammeus (but not the melanocytosis) was bilateral in three of seven cases. Choroidal melanoma was observed in three patients and an optic disc melanocytoma in one. (For a more complete description of this recondite subject 8 beyond what is offered in the textbook one should consult Shields et al. Arch Ophthalmol 2011; 129:746–750.) Topical drug treatment for ocular surface tumors as well as plaque brachytherapy are critically assessed and introduced for the first time to the textbook, as is an exploration of the controversial role of sentinel lymph node biopsy and dissection for conjunctival melanoma. Especially helpful for clinicians and pathologists are demystifying descriptions of the clinical and pathologic features, as well as management options, of the challenging entities of primary acquired melanosis, emergent melanoma, and sebaceous carcinoma. Propranolol for promoting regression of eyelid capillary hemangioma is included among the different approaches for this nettlesome problem which can cause amblyopia. An excellent description of the management of malignant lacrimal gland epithelial tumors is offered. Finally, particularly insightful and representative of the contemporary sophistication of the authors is the application of the World Health Organization (WHO) classification of lymphomas for ocular adnexal lesions and the American Joint Commission on Cancer (AJCC) classification of ocular adnexal nonlymphomatous solid tumors. What makes this textbook a distinctively authoritative resource is the combination of the unrivaled clinical experience of Carol and Jerry Shields as practicing clinical ophthalmic oncologists and their intimate familiarity with the appropriate literature. They have earned the accolade of being the ultimate “power couple” of American ophthalmology and ophthalmic oncology. Their human side, however, has not been adversely affected by their devotion to their professional calling. They have raised a marvelous family with most of their well-adjusted children now launched into adulthood. The Shields are a national, and indeed an international, treasure who have helped untold numbers of patients and who have generously and personally shared their knowledge with colleagues and trainees, and also prodigiously enriched the literature with their high-quality publications based on the codification of their clinical experience. I admire them greatly and am a devoted aficionado of this textbook, to which I frequently refer for digestible summaries of unusual or arcane topics. I wish them many more years of productivity as they persist in their labors to further develop and advance ophthalmic oncology. Frederick A. Jakobiec, MD, DSc Henry Willard Williams Professor Emeritus of Ophthalmology and Pathology Harvard Medical School Former Chairman of Ophthalmology Harvard Medical School Former Chief of Ophthalmology Massachusetts Eye and Ear Infirmary Current Director David Glendenning Cogan Laboratory of Ophthalmic Pathology Massachusetts Eye & Ear Infirmary, Boston, Massachusetts 9 PREFACE Forty years in the management of eyelid, conjunctival, and orbital tumors Forty years is a long time. Forty years is beyond the time spent in most careers. Forty years represents the number of years that we have devoted our medical and surgical practice to the study of eyelid, conjunctival, and orbital tumors. We have focused our career on the topic of periocular tumors, benign or malignant, and the numerous simulating lesions. Every working day, we have traveled from our small farm to the city of Philadelphia and studied and helped patients with periocular tumors, providing diagnoses, treatments, and reconstructions. We have spent precious time exploring new and progressive treatments to maximize patient comfort, safety, and cosmesis. After all is done, then we traveled home in the early evening. Forty years represents the time that we used to discover, think, design, critique, and perform hours-on-end research that culminated in numerous ocular oncology projects with information pushing the field forward. Slow and steady progress, but looking back, we participated in giant leaps of knowledge. In 1999, we published our first series of atlases in three volumes, entitled Atlas of Eyelid and Conjunctival Tumors, Atlas of Intraocular Tumors, and Atlas of Orbital Tumors. Following numerous enhancements and updates, we subsequently wrote a second edition in two volumes in 2008 entitled Intraocular Tumors: An Atlas and Textbook and Eyelid, Conjunctival, and Orbital Tumors: An Atlas and Textbook. We now provide you with the third edition of our atlases. This volume is embellished with new illustrations, updated references and text, improved imaging modalities, and novel observations. We have carefully planned the layout of this book so that it is reader-friendly with six illustrations per page to tell a story or make a clinical or surgical point. Each diagnostic entity is described in anatomical order based on eyelid then conjunctiva then orbital tissue. Go ahead and flip through to enjoy the full experience. This book is generously illustrated with numerous images of common lesions, such as chalazion and pinguecula, as well as rare and fascinating conditions like lipoid proteinosis of the eyelid, ligneous conjunctivitis, orbital juvenile xanthogranuloma, and conjunctival hereditary benign intraepithelial dyskeratosis. This atlas is overflowing in clinicopathologic correlations and clinical “pearls” based on our personal experience. Surgical principles are illustrated with high-quality professional drawings and photographs. We hope that this unique textbook and atlas will benefit residents and fellows in ophthalmology, general ophthalmologists, specialists in external disease, oculoplastic surgery, and ophthalmic pathology, as well as other practitioners. We believe the reader will find it useful in clinical practice and enjoyable to read. Jerry A. Shields, MD Carol L. Shields, MD 10