Commentary The Pediatrician
and the Care of Children
Most of us, as general pediatricians, know little about the technical aspects of modern care for the child with neoplasia. As Dr Pizzo points out in the commentary which follows, this is one of the disease groups for which this technical care is best given at a tertiary care center. However,
the general pediatrician must make the diagnosis promptly, refer the patient to the appropriate site, maintain contact with the child and family, deal with intercurrent illnesses, and keep up-to-date with modern advances regarding the diagnosis and treatment
Cancer
and the Pediatrician:
Although ing causes
approximately 6550 new cases are diagnosed each year in children less than 1 5 years of age in the United States. Thus, most pediatricians are unlikely to diagnose cancer in a child in their clinical practice. At the same
time, major progress in the treatment of children with cancer has been made in the last decades. Indeed, more than 50% to 60% of children diagnosed with cancer are likely to become prolonged survivors, and most will be cured of their malignancy. Accordingly, it has been estimated that in 1990, 1 of every 1000 young adults reaching the age of 20 will be a survivor of childhood cancer.
As many as 40% of cancer survivors will
have
some
disability
either from their cancer ment,
and
treatment
they
may
resulting
or its treatseek
for this secondary
of these disorders to be able to interpret them to the family. Hematology/oncology is one to the Topics for Review in the 1990-91 volume of Pediatrics in Review. The two commentaries that follow provide a good overview of the pediatrician’s role in the care of children with malignancy. Dr Pizzo, a member of the Editorial Board, has summarized the current status of diagnosis and treatment of children with malignancies. Dr Sahler, editor of the PREP self-
assessment
examination
tive member
of the program
An Evolving
cancer is among the leadof death in children, only
medical
disabil-
With Malignancy
difficulties
and an ac-
these
patients
may
Second,
bring
the
principles of cancer biology and treatment that have been generated in pediatric oncology serve as the cornerstone for approaching adult malignancies. Moreover, knowledge about pediatric oncology also permits pediatricians to keep pace with devel-
opments in molecular biology and genetics and with the incorporation of biotechnology into diagnosis and treatment. Third, because many of the cancers in children have become treatable, it is important that children receive the most advanced therapy possible. This mandates that the diagnosis be determined quickly and correctly and that children be referred to centers where appropriate therapy can be obtained. At the same time, since children are likely to survive, it is also imperative that they remain connected to their home, school, and community.
Thus, although cancer in children might be viewed as a rare disease (it
likely to play a pivotal both as a participant
accounts of only 2% of all cancers), its successful treatment makes it increasingly important to the pediatric
treatment protocol and as a support to and community advocate for the child and family. For all of these rea-
and medical community for a number of reasons. First, in the future, an increasing number of physicians will have contact with individuals who were treated for cancer during childhood. Understanding the biology of childhood cancer and the treatment
sons, knowledge about the advances in the treatment of children with cancer are more important than ever. Several common threads comprise
regimens
that
are
currently
being
at the
University
of
tions. During the coming PREP 2 Year 6, we will publish a number of other articles pertaining to specific
-
malignancies in children. The care of children with malignancies must be a joint effort involving the pediatric generalist and the tertiary care center. These commentaries, plus cles which will follow, should this relationship. R.J.H.
the artifacilitate
in behav-
ity.
used will permit the pediatrician to evaluate some of the problems and
pediatrics
Rochester, has presented succinctly what we now know about caring for the child and family in these situa-
Partnership
to his or her attention.
the fabric
ioral
The
child’s
of pediatric
pediatrician
is
role in serving in the child’s
oncology.
First,
advances in biology and molecular genetics have highlighted the heterogeneity of childhood cancer. For example, while acute lymphoblastic leukemia is the most common childhood
cancer, accounting for 30% of newly diagnosed malignancies, it can be divided into low- and high-risk groups on the basis of the child’s age at diagnosis, initial white blood cell count, and an increasing number of biological
markers
morphology, togenetics,
(eg,
lymphoblast
immunophenotype, cybiochemical characteris-
tics) that delineate
both
the patient’s
prognosis and treatment. As the refinement of the diagnostic repertoire improves, it becomes possible to stage patients not only on the basis of the traditional microscopic appearance of their abnormal cells or tumors but also
on the basis
of their
immu-
nocytochemistry and molecular genetics. Because treatment decisions hinge increasingly on a composite of clinical
and
laboratory
studies,
it is
also important for the tissue or bone marrow samples to be processed correctly and triaged to the appropnate laboratory services. Thus, when confronted
cer
with
a child
is suspected,
would
benefit
in whom
the
from
can-
pediatrician
discussion
with
a
pediatric oncologist about optimal procedures for obtaining and processing diagnostic materials. A second shared element is the important
role
that
pediatric
have played in elucidating cepts
in tumor
biology.
tumors
novel conWhether
in
the use of monoclonal antibodies (eg, CALLA) for the characterization of leukemias, the recognition of oncogenes in tumongenesis (eg, c-myc in Burkitt’s
lymphoma)
the
pediatrics in review #{149} vol. 12 no. 1 july 1990 Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
staging PIR
of 5
Pediatric
Oncology
disease
and outcome
neuroblastoma),
(eg, N-myc importance
the
in of
genetics in the vertical transmission of cancer (retinoblastoma) or in the elucidation of antioncogenes or suppressor genes (Rb gene), pediatric oncology has served as a focal point for understanding the fundamentals
of cancer. Indeed, the principles uncovered in the study of pediatric neoplasms have served as the foundation for studies
being
extended
to the
team that often oncologist, pist, clinical
includes
ers, psychologist, clergy,
a pediatric
surgeon, radiation pharmacist, social
and
nurses,
physical,
therawork-
teachers,
occupational
and recreational therapists. The goal of pediatric oncology treat
the
tumor
preserve
successfully
the integrity
is to and
to
and well-being
of the child. Because such treatment is complex and comprehensive, it is nearly always administered best in a center devoted to the care of children with cancer. Not only does this guar-
cancers of adults. Even the integral role of cancer and the immune systern that has emerged with the as-
antee the best possible
sociation of Kaposi’s sarcoma and lymphomas in adults with acquired immunodeficiency syndrome had its biological precedent in children with
child and family, but also the basis for future progress. In the United States, most university-based hospitals have a special
congenital immunodeficiencies who developed lymphoproliferative disorders or lymphomas. A third common thread is that vir-
program participate cooperative
tually
Children’s
all pediatric
systemic tumor,
malignancies
diseases. for example,
are
in pediatric in one groups
National
Cancer
the Pediatric
appears
over
localized
60%
oncology
of the two sponsored
Institute.
Cancer
Even when a solid
care for the
In fact, the
Study
Oncology
of children
and major by the
Group
Group with
and
enroll
cancer
in
to an organ or site, the potential that microscopic metastases are already
the United States treatment protocols.
present is nearly always the case. fact, the initial utility of chemotherapy
studies provide state-of-the-art care and offer the prospect for improving treatment in the future. Referral of children to centers participating in treatment protocols is the best way for the pediatrician to assure that his patient is receiving optimal evaluation and treatment. Information about which treatment centers provide care for the specific diagnosis of the patient can be obtained from the Na-
was shown in childhood Wilms
tumor,
and
In
leukemia
and
concept
of
the
combination therapy (eg, regimens including the prophylaxis of sanctuary sites, such as the central nervous system) also began with childhood cancer.
The combination
of surgery,
radiation therapy, and chemotherapy has become standard in the treatment of a number of childhood cancers. Because survival is now expected for children with cancer, a multidisciplinary
approach
is integral
to their
treatment and must include psychosocial support for the child and family. Indeed,
the
treatment
cancer has become in using
of childhood
highly specialized
the services
and
skills
of a
diverse number of health care professionals. Such care is built upon a
tional based
Cancer national
referred through
Institute computerinformation system,
to as PDQ and 1-800-4-CANCER.
history
of
173
patients
with
leukemia
treated
supportively
PIR 6
pediatrics in review
acute
at Me#{149}
accessible
providing that links
their care, it is important with their home and com-
munity be maintained. The child’s pediatrician can provide an integral role by providing coordinated care with the cancer
treatment
morial
Center
center
and
by
vol. 12 no. 1 july
acquisition
of this
knowledge
represents an opportunity for the pediatrician to learn not only about one of the success stories of modern
medicine future
but also to glimpse
of medical
into the
research.
The opportunity to apply the fruits of biomedical research to the care of children with developments
cancer is exciting. The in genetic engineering
are producing regents that can regulate the patient’s immune system to control tumor proliferation vent or attenuate some effects that accompany
and to preof the side the use of
cytotoxic chemotherapy. In the immediate future, the evolving understanding of the genetic basis of cancer will offer modalities to control the growth
and
differentiation
of tumor
cells and, hopefully, will provide safer and more selective approaches to the effective and successful treatment of cancer in children.
National
Cancer
Institute
National Institutes of Health Bethesda, Maryland REFERENCES 1. Pizzo
PA, Poplack,
Practice
DG,
of Pediatric
eds. Principles and Oncology. Philadel-
phia, PA: J. B. Lippincott
Co; 1989
Lessons
for Cancer
and
Allied
Diseases in New York City from 1926 to 1948. The study population included
The
Philip A. Pizzo, MD Pediatric Branch
Because children with cancer frequently have to travel to the center
Caring for the Child with Cancer and the Family: Learned From Children With Acute Leukemia In a report appearing in 1951, Southam et al1 reported on the natural
into cooperative These treatment
serving as a resource to the child’s family and community. This might involve treating iatrogenic complications, such as fever and neutropenia, or monitoring the patient’s blood counts and transfusion requirements. In some cases, pediatricians may become involved in administering parts of the treatment protocol and in serving as an information resource for the child’s parents, siblings, friends, and community. Each of the responsibilities mandates that the pediatrician be well-informed and knowledgeable about the treatment advances which have occurred in pediatric oncology.
65 children
less than
10 years
of age. The average of these patients
length of survival was 19.3 weeks
after the onset of symptoms attributable to leukemia. Of the entire sam-
1990
Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
Cancer and the Pediatrician: An Evolving Partnership Philip A. Pizzo Pediatrics in Review 1990;12;5 DOI: 10.1542/pir.12-1-5-a
Updated Information & Services
including high resolution figures, can be found at: http://pedsinreview.aappublications.org/content/12/1/5.2
Permissions & Licensing
Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://pedsinreview.aappublications.org/site/misc/Permissions.xhtml
Reprints
Information about ordering reprints can be found online: http://pedsinreview.aappublications.org/site/misc/reprints.xhtml
Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
Cancer and the Pediatrician: An Evolving Partnership Philip A. Pizzo Pediatrics in Review 1990;12;5 DOI: 10.1542/pir.12-1-5-a
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pedsinreview.aappublications.org/content/12/1/5.2
Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 1990 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.
Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
and the Care of Children
Most of us, as general pediatricians, know little about the technical aspects of modern care for the child with neoplasia. As Dr Pizzo points out in the commentary which follows, this is one of the disease groups for which this technical care is best given at a tertiary care center. However,
the general pediatrician must make the diagnosis promptly, refer the patient to the appropriate site, maintain contact with the child and family, deal with intercurrent illnesses, and keep up-to-date with modern advances regarding the diagnosis and treatment
Cancer
and the Pediatrician:
Although ing causes
approximately 6550 new cases are diagnosed each year in children less than 1 5 years of age in the United States. Thus, most pediatricians are unlikely to diagnose cancer in a child in their clinical practice. At the same
time, major progress in the treatment of children with cancer has been made in the last decades. Indeed, more than 50% to 60% of children diagnosed with cancer are likely to become prolonged survivors, and most will be cured of their malignancy. Accordingly, it has been estimated that in 1990, 1 of every 1000 young adults reaching the age of 20 will be a survivor of childhood cancer.
As many as 40% of cancer survivors will
have
some
disability
either from their cancer ment,
and
treatment
they
may
resulting
or its treatseek
for this secondary
of these disorders to be able to interpret them to the family. Hematology/oncology is one to the Topics for Review in the 1990-91 volume of Pediatrics in Review. The two commentaries that follow provide a good overview of the pediatrician’s role in the care of children with malignancy. Dr Pizzo, a member of the Editorial Board, has summarized the current status of diagnosis and treatment of children with malignancies. Dr Sahler, editor of the PREP self-
assessment
examination
tive member
of the program
An Evolving
cancer is among the leadof death in children, only
medical
disabil-
With Malignancy
difficulties
and an ac-
these
patients
may
Second,
bring
the
principles of cancer biology and treatment that have been generated in pediatric oncology serve as the cornerstone for approaching adult malignancies. Moreover, knowledge about pediatric oncology also permits pediatricians to keep pace with devel-
opments in molecular biology and genetics and with the incorporation of biotechnology into diagnosis and treatment. Third, because many of the cancers in children have become treatable, it is important that children receive the most advanced therapy possible. This mandates that the diagnosis be determined quickly and correctly and that children be referred to centers where appropriate therapy can be obtained. At the same time, since children are likely to survive, it is also imperative that they remain connected to their home, school, and community.
Thus, although cancer in children might be viewed as a rare disease (it
likely to play a pivotal both as a participant
accounts of only 2% of all cancers), its successful treatment makes it increasingly important to the pediatric
treatment protocol and as a support to and community advocate for the child and family. For all of these rea-
and medical community for a number of reasons. First, in the future, an increasing number of physicians will have contact with individuals who were treated for cancer during childhood. Understanding the biology of childhood cancer and the treatment
sons, knowledge about the advances in the treatment of children with cancer are more important than ever. Several common threads comprise
regimens
that
are
currently
being
at the
University
of
tions. During the coming PREP 2 Year 6, we will publish a number of other articles pertaining to specific
-
malignancies in children. The care of children with malignancies must be a joint effort involving the pediatric generalist and the tertiary care center. These commentaries, plus cles which will follow, should this relationship. R.J.H.
the artifacilitate
in behav-
ity.
used will permit the pediatrician to evaluate some of the problems and
pediatrics
Rochester, has presented succinctly what we now know about caring for the child and family in these situa-
Partnership
to his or her attention.
the fabric
ioral
The
child’s
of pediatric
pediatrician
is
role in serving in the child’s
oncology.
First,
advances in biology and molecular genetics have highlighted the heterogeneity of childhood cancer. For example, while acute lymphoblastic leukemia is the most common childhood
cancer, accounting for 30% of newly diagnosed malignancies, it can be divided into low- and high-risk groups on the basis of the child’s age at diagnosis, initial white blood cell count, and an increasing number of biological
markers
morphology, togenetics,
(eg,
lymphoblast
immunophenotype, cybiochemical characteris-
tics) that delineate
both
the patient’s
prognosis and treatment. As the refinement of the diagnostic repertoire improves, it becomes possible to stage patients not only on the basis of the traditional microscopic appearance of their abnormal cells or tumors but also
on the basis
of their
immu-
nocytochemistry and molecular genetics. Because treatment decisions hinge increasingly on a composite of clinical
and
laboratory
studies,
it is
also important for the tissue or bone marrow samples to be processed correctly and triaged to the appropnate laboratory services. Thus, when confronted
cer
with
a child
is suspected,
would
benefit
in whom
the
from
can-
pediatrician
discussion
with
a
pediatric oncologist about optimal procedures for obtaining and processing diagnostic materials. A second shared element is the important
role
that
pediatric
have played in elucidating cepts
in tumor
biology.
tumors
novel conWhether
in
the use of monoclonal antibodies (eg, CALLA) for the characterization of leukemias, the recognition of oncogenes in tumongenesis (eg, c-myc in Burkitt’s
lymphoma)
the
pediatrics in review #{149} vol. 12 no. 1 july 1990 Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
staging PIR
of 5
Pediatric
Oncology
disease
and outcome
neuroblastoma),
(eg, N-myc importance
the
in of
genetics in the vertical transmission of cancer (retinoblastoma) or in the elucidation of antioncogenes or suppressor genes (Rb gene), pediatric oncology has served as a focal point for understanding the fundamentals
of cancer. Indeed, the principles uncovered in the study of pediatric neoplasms have served as the foundation for studies
being
extended
to the
team that often oncologist, pist, clinical
includes
ers, psychologist, clergy,
a pediatric
surgeon, radiation pharmacist, social
and
nurses,
physical,
therawork-
teachers,
occupational
and recreational therapists. The goal of pediatric oncology treat
the
tumor
preserve
successfully
the integrity
is to and
to
and well-being
of the child. Because such treatment is complex and comprehensive, it is nearly always administered best in a center devoted to the care of children with cancer. Not only does this guar-
cancers of adults. Even the integral role of cancer and the immune systern that has emerged with the as-
antee the best possible
sociation of Kaposi’s sarcoma and lymphomas in adults with acquired immunodeficiency syndrome had its biological precedent in children with
child and family, but also the basis for future progress. In the United States, most university-based hospitals have a special
congenital immunodeficiencies who developed lymphoproliferative disorders or lymphomas. A third common thread is that vir-
program participate cooperative
tually
Children’s
all pediatric
systemic tumor,
malignancies
diseases. for example,
are
in pediatric in one groups
National
Cancer
the Pediatric
appears
over
localized
60%
oncology
of the two sponsored
Institute.
Cancer
Even when a solid
care for the
In fact, the
Study
Oncology
of children
and major by the
Group
Group with
and
enroll
cancer
in
to an organ or site, the potential that microscopic metastases are already
the United States treatment protocols.
present is nearly always the case. fact, the initial utility of chemotherapy
studies provide state-of-the-art care and offer the prospect for improving treatment in the future. Referral of children to centers participating in treatment protocols is the best way for the pediatrician to assure that his patient is receiving optimal evaluation and treatment. Information about which treatment centers provide care for the specific diagnosis of the patient can be obtained from the Na-
was shown in childhood Wilms
tumor,
and
In
leukemia
and
concept
of
the
combination therapy (eg, regimens including the prophylaxis of sanctuary sites, such as the central nervous system) also began with childhood cancer.
The combination
of surgery,
radiation therapy, and chemotherapy has become standard in the treatment of a number of childhood cancers. Because survival is now expected for children with cancer, a multidisciplinary
approach
is integral
to their
treatment and must include psychosocial support for the child and family. Indeed,
the
treatment
cancer has become in using
of childhood
highly specialized
the services
and
skills
of a
diverse number of health care professionals. Such care is built upon a
tional based
Cancer national
referred through
Institute computerinformation system,
to as PDQ and 1-800-4-CANCER.
history
of
173
patients
with
leukemia
treated
supportively
PIR 6
pediatrics in review
acute
at Me#{149}
accessible
providing that links
their care, it is important with their home and com-
munity be maintained. The child’s pediatrician can provide an integral role by providing coordinated care with the cancer
treatment
morial
Center
center
and
by
vol. 12 no. 1 july
acquisition
of this
knowledge
represents an opportunity for the pediatrician to learn not only about one of the success stories of modern
medicine future
but also to glimpse
of medical
into the
research.
The opportunity to apply the fruits of biomedical research to the care of children with developments
cancer is exciting. The in genetic engineering
are producing regents that can regulate the patient’s immune system to control tumor proliferation vent or attenuate some effects that accompany
and to preof the side the use of
cytotoxic chemotherapy. In the immediate future, the evolving understanding of the genetic basis of cancer will offer modalities to control the growth
and
differentiation
of tumor
cells and, hopefully, will provide safer and more selective approaches to the effective and successful treatment of cancer in children.
National
Cancer
Institute
National Institutes of Health Bethesda, Maryland REFERENCES 1. Pizzo
PA, Poplack,
Practice
DG,
of Pediatric
eds. Principles and Oncology. Philadel-
phia, PA: J. B. Lippincott
Co; 1989
Lessons
for Cancer
and
Allied
Diseases in New York City from 1926 to 1948. The study population included
The
Philip A. Pizzo, MD Pediatric Branch
Because children with cancer frequently have to travel to the center
Caring for the Child with Cancer and the Family: Learned From Children With Acute Leukemia In a report appearing in 1951, Southam et al1 reported on the natural
into cooperative These treatment
serving as a resource to the child’s family and community. This might involve treating iatrogenic complications, such as fever and neutropenia, or monitoring the patient’s blood counts and transfusion requirements. In some cases, pediatricians may become involved in administering parts of the treatment protocol and in serving as an information resource for the child’s parents, siblings, friends, and community. Each of the responsibilities mandates that the pediatrician be well-informed and knowledgeable about the treatment advances which have occurred in pediatric oncology.
65 children
less than
10 years
of age. The average of these patients
length of survival was 19.3 weeks
after the onset of symptoms attributable to leukemia. Of the entire sam-
1990
Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
Cancer and the Pediatrician: An Evolving Partnership Philip A. Pizzo Pediatrics in Review 1990;12;5 DOI: 10.1542/pir.12-1-5-a
Updated Information & Services
including high resolution figures, can be found at: http://pedsinreview.aappublications.org/content/12/1/5.2
Permissions & Licensing
Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://pedsinreview.aappublications.org/site/misc/Permissions.xhtml
Reprints
Information about ordering reprints can be found online: http://pedsinreview.aappublications.org/site/misc/reprints.xhtml
Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
Cancer and the Pediatrician: An Evolving Partnership Philip A. Pizzo Pediatrics in Review 1990;12;5 DOI: 10.1542/pir.12-1-5-a
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pedsinreview.aappublications.org/content/12/1/5.2
Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1979. Pediatrics in Review is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 1990 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0191-9601.
Downloaded from http://pedsinreview.aappublications.org/ at University of California San Diego on June 9, 2015
