Table Of ContentTumors
of the Fetus
and Infant
Springer
New York
Berlin
Heidelberg
Barcelona
Hong Kong
London
Milan
Paris
Singapore
Tokyo
Hart Isaacs, Jr., MD
Associate Pathologist, Department of Pathology
Children’s Hospital San Diego, and
Associate Clinical Professor of Pathology
University of California, San Diego School of Medicine
San Diego, California
Tumors
of the Fetus
and Infant
An Atlas
With 208 Figures in 585 Parts
Hart Isaacs, Jr., MD
Associate Pathologist
Department of Pathology
Children’s Hospital San Diego
Associate Clinical Professor of Pathology
University of California, San Diego School of Medicine
San Diego, CA 92123-4282, USA
[email protected]
Library of Congress Cataloging-in-Publication Data
Isaacs, Hart.
Tumors of the fetus & infant : an atlas / Hart Isaacs, Jr.
p. ; cm.
Includes bibliographical references and index.
ISBN 0-387-95186-5 (alk. paper)
1. Tumors in children—Atlases. 2. Infants—Diseases—Atlases.
3. Fetus—Diseases—Atlases. I. Title: Tumors of the fetus and infant. II. Title.
[DNLM: 1. Neoplasms—Infant—Atlases. 2. Neoplasms—embryology—Atlases. QZ 17
I73t 2002]
RC281.C4 I727 2002
618.92'992—dc21 2001054922
Printed on acid-free paper.
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To my family,
without whose support
this atlas would not
have been possible
Preface
This atlas attempts to bring together in a simple and clear fashion the wealth of in-
formation presently available concerning neoplasia in the young. The purpose is to
present a concise account, which will serve as the basis for a useful teaching aid,
of the clinical and pathological features of neoplastic disease and tumorlike condi-
tions in the fetus and infant.
Throughout I have used my own material as fully as possible and have introduced
accounts of personally studied cases supplemented by ones from other colleagues
illustrative of the subjects under discussion. For the same reason, I make frequent
reference to my own published articles and to earlier publications, Tumors of the
Fetus and Newborn, and the chapter entitled Tumors in Potter’s Pathology of the
Fetus and Infant(Refs. 1 and 2 of Chapter 1), the contents of which in many ways
supplement the present work. Most photographs and tables have been produced from
personally studied material, while others were taken, by permission, from various
articles appearing in the literature.
The atlas is addressed primarily to pathologists, but I hope that clinicians, resi-
dents, and medical students in pathology and other medical specialties may find it
useful and beneficial as a concise, easy-to-use, quick ready reference source.
HART ISAACS, JR., MD
vii
Acknowledgments
My grateful thanks are due to my colleagues and friends who have assisted me in
various ways during the preparation of this book, especially to all who have allowed
me to use illustrations from their published work and to the publishers of journals
and books from which they have been produced. Particular thanks is due to Dr.
Henry Krous of the Department of Pathology, Children’s Hospital, San Diego, for
his continual encouragement and support. The help given me with the electron pho-
tomicrographs by Ann Peters of the Department of Pathology, Children’s Hospital,
San Diego, has been invaluable. I want also to thank my publishers for their help-
fulness in the preparation and production of this atlas.
HART ISAACS, JR., MD
ix
Contents
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii
Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix
1 General Survey. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
2 Germ Cell Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
3 Soft Tissue Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . 37
4 Tumors and Tumorlike Conditions of the Skin . . . . . . . . 113
5 Neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137
6 Leukemia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
7 Histiocytoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181
8 Brain Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199
9 Tumors of the Eye . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241
10 Renal Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261
11 Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303
12 Adrenocortical Tumors . . . . . . . . . . . . . . . . . . . . . . . . . 337
xi
xii Contents
13 Pancreatic Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353
14 Salivary Gland Tumors . . . . . . . . . . . . . . . . . . . . . . . . . 367
15 Cardiac Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377
16 Tumors of the Lung . . . . . . . . . . . . . . . . . . . . . . . . . . . 387
17 Tumors and Tumorlike Conditions of Bone. . . . . . . . . . . 407
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425
1
General Survey
Although tumors and tumorlike conditions of the fetus and ing an understanding of the prenatal natural history and patho-
infant occur infrequently, they present challenging diagnostic physiology of these tumors [12,16,17]. Advances in imaging
and treatment problems. There is considerable drama and ter- techniques, particularly ultrasonography and rapid magnetic
rible parental apprehension when dealing with a tumor in an resonance imaging, are responsible for making an early di-
infant or unborn child. Unfamiliarity with these conditions agnosis possible. Tumors most often diagnosed by antenatal
may lead to an erroneous diagnosis and unnecessarily ag- ultrasonography are teratomas, mesoblastic nephroma, liver
gressive therapy. Neoplasms and tumorlike conditions occur- tumors, and neuroblastoma [16,17].
ring during the first year of life are not entirely the same as
those observed in the adolescent or adult—the types, inci-
Tumors Occurring in Patients
dence, and clinical features differ, as do behavior and response
with Congenital Malformations
to treatment [1–14] (Tables 1.1–1.3).
No tumor of the adult grows as rapidly as does the normal and Syndromes
embryo. In the early stages of development, normal embry-
onic cells may have some of the characteristics of neoplastic
An important relationship exists between certain congenital
cells. Generally it is accepted by pathologists that the diag-
malformations and syndromes and the development of neo-
nosis of malignancy depends on certain microscopic criteria,
plasms [1,10,11,18–24] (Table 1.4). In this setting the tumor
but these are not always helpful or valid in a young child.
occurs after birth or later in life in individuals with specific
Normal developing organs and tissues display significant mi-
inherited diseases, or congenital anomalies or malformation
totic activity and contain immature or embryonic-appearing
syndromes, many of whom probably have an underlying
structures that may simulate malignancy. It is often only from
chromosomal defect (Table 1.5).
knowledge of the postnatal course of a tumor that definite
Hereditary conditions such as the neurocutaneous syn-
proof can be obtained of its malignancy [2].
dromes, also known as “phakomatoses,” are characterized by
Tumors that are not histologically malignant may cause
a high incidence of neoplasia. For example, tuberous sclero-
death in utero or shortly after birth because of their location—
sis and neurofibromatosis, which are autosomal dominant, pre-
for example, a large lymphangioma, mature teratoma, or fi-
dispose the individual to the development of gliomas and ma-
bromatosis involving vital structures in the neck or medi-
lignant peripheral nerve tumors [20,21] (Tables 1.4 and 1.5).
astinum [2].
Tumors occurring in the perinatal period differ markedly
Incidence
in their presenting clinical signs and symptoms from those
found in older individuals (Tables 1.3 and 1.4) [1,10,11].
Maternal dystocia may be the first sign of a large-space- Tumors of the newborn are uncommon, with an incidence of
occupying congenital tumor such as a giant intracranial or 7.2 in 100,000 live births in the United Kingdom [3]. Most
sacrococcygeal teratoma. Fetal hydrops is another unique fetal and newborn tumors are benign rather than malignant,
manifestation [15]. Rupture during delivery of a large tumor, with cancer estimated to occur in 3.65 of 100,000 live births
such as a neuroblastoma or a hepatoblastoma, with fatal in the United States [13]. Malignant tumors, although rare,
exsanguination of the fetus, is a dramatic illustration of an- are an important cause of perinatal mortality.
other unusual presentation. Several series indicate that the most common malignancies
The antenatal diagnosis of fetal neoplasms is one aspect of noted at birth are neuroblastoma, leukemia, brain tumors, and
fetal disease that only recently has been implemented, allow- sarcomas in that order [3,4,7,8,11,13] (Table 1.1). Almost all
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