Table Of Content2
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
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Third edition
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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
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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
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verification of medical diagnoses, indications, appropriate pharmaceutical selections and dosages, and treatment
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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.
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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
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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
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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
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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
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