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Pediatric Head and Neck Malignancies
US Incidence and Trends Over 2 Decades
James T. Albright, MD;
Alan K. Topham, BA;
James S. Reilly, MD
Arch Otolaryngol Head Neck Surg. 2002;128:655-659.
ABSTRACT
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Background Recent reports have noted an increase in the overall incidence of pediatric
cancer.
Objective To determine whether this trend is applicable to malignancies of the
head and neck in children.
Design Using the National Cancer Institute's Surveillance, Epidemiology, and
End Results tumor database, we determined the incidence of all cancers diagnosed
from 1973 through 1996 in children younger than 19 years. This was compared
with the incidence of head and neck malignancies within the same population.
Rates were then determined for eight 3-year periods from 1973-1975 to 1994-1996
and adjusted by use of three 5-year age groups weighted by the 1970 US standard
population.
Results A total of 24 960 malignancies diagnosed in children younger than
19 years were identified for the study period. From this group, 3050 tumors
(12%) were located in the head and neck. The average annual rate of all cancer
(number of malignancies per 100 000 person-years) in children younger
than 15 years rose 25% from 11.22 (95% confidence interval [CI], 10.70-11.74)
for 1973-1975 to 14.03 (95% CI, 13.46-14.60) for 1994-1996. Among malignancies
of the head and neck, the incidence rate increased 35% from 1.10 (95% CI,
0.94-1.26) to 1.49 (95% CI, 1.30-1.68) during the same period.
Conclusion The incidence of head and neck malignancies among children younger than
15 years in the United States from 1973 through 1996 increased at a greater
rate than childhood cancer in general.
INTRODUCTION
CANCER REPRESENTS a significant cause of mortality among the pediatric
population. The frequency with which children between the ages of 5 and 14
years die from cancer is second only to death from accidental trauma.1 Despite improvements in managing many pediatric malignancies,
the incidence of childhood cancer continues to rise. Over the past 2 decades,
annual increases of 1% to 2% in the rate of pediatric malignancies have been
reported in the United States.2-5
Similar increases have been reported in Australia, Spain, and Britain.6-8
Whether this rising trend applies to pediatric head and neck cancer
remains unknown. Pediatric oncology reports often lack specific information
regarding the incidence and trends of head and neck cancer. Past reviews of
this population have been limited to single-institution studies, which has
made meaningful interpretation of long-term trends difficult.9-12
To determine if the rate of pediatric head and neck cancer has increased in
the United States, we performed a search of the National Cancer Institute's
Surveillance, Epidemiology, and End Results (SEER) tumor registry.
PATIENTS AND METHODS
The SEER database is a large, population-based tumor registry maintained
by the National Cancer Institute since 1973. The geographic areas included
are diverse, encompassing the states of Connecticut, Hawaii, Iowa, New Mexico,
and Utah as well as the metropolitan areas of Atlanta, Ga, Detroit, Mich,
Los Angeles, Calif, San Francisco-Oakland, Calif, and Seattle-Puget Sound,
Wash. The SEER database represents an estimated 10% of the US population.
All malignant neoplasms, with the exception of squamous and basal cell skin
cancers, are included within the database. Data are available through special
public access.13
For our study, all cases of cancer newly diagnosed from 1973 through
1996 in children younger than 19 years were identified. Head and neck malignancies
were selected using appropriate codes from the International
Classification of Diseases for Oncology, 2nd Edition.14
Tumors of the central nervous system were excluded. Descriptive statistics
were compiled for each patient's age at diagnosis, sex, and tumor histologic
characteristics.
Incidence rates for those children aged 14 years or younger were determined
for eight 3-year periods from 1973-1975 to 1994-1996. Three-year means reduced
the potential for annual variation. The US Bureau of the Census supplied annual
population estimates. All rates were then adjusted by use of 5-year age groups
weighted by the 1970 US standard population. Incidence rates were expressed
in malignancies per 100 000 person-years. Because the US Bureau of the
Census provides adjustment data in 5-year age groupings, we were compelled
to designate 14 years rather than 18 years as the top age limit for this analysis.
Male and female incidence rates for each cohort were also determined. Standard
errors and the resulting 95% confidence intervals (CIs) were calculated according
to the method described by Keyfitz.15
RESULTS
DEMOGRAPHICS
A total of 24 960 malignancies diagnosed in children younger than
19 years were identified through the SEER database from 1973 through 1996.
This group was made up of 12 369 boys (49.6%) and 12 591 girls (50.4%),
with a mean age of 9.6 years.
Among this population, 3050 tumors (12%) were located in the head and
neck. Primary tumors represented 3026 (99.2%) of the total number while an
additional 24 (0.8%) were cases of second malignant neoplasms. Head and neck
tumors were found in 1432 boys (47%) and 1618 girls (53%), with a slightly
higher mean age of 10.4 years. The higher proportion of girls seen among the
head and neck group was statistically significant (P<.05)
when compared with the overall cancer population. Reported cases of head and
neck cancer were most frequent among teenagers 15 to 18 years old (39%), followed
by children 4 years or younger (27%), 10 to 14 years (21%), and 5 to 9 years
(13%). Figure 1 illustrates the
age distribution of pediatric head and neck malignancies.
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Figure 1. Patient ages at diagnosis of pediatric
head and neck cancer.
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TUMOR PATHOLOGIC CHARACTERISTICS AND DISTRIBUTION
There was a diverse range of tumor pathologic characteristics in the
pediatric head and neck cancer population (Table 1). Taken as a whole, lymphoma involving the head and neck
was the most common cancer type (27%), followed by neural tumors (23%), thyroid
malignancies (21%), and soft tissue sarcomas (12%). Papillary thyroid carcinoma
(18%) was the leading pathologic diagnosis. Other frequent malignancies included
Hodgkin lymphoma (17%), retinoblastoma (16%), non-Hodgkin lymphoma (10%),
and rhabdomyosarcoma (8%). Unlike adult head and neck cancer, pediatric cases
of squamous cell carcinoma were rare, occurring in fewer than 2% of all cases.
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Table 1. Pediatric Head and Neck Tumor Histologic Characteristics
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Age differences were noted. Neural tumors and soft tissue sarcomas remained
more prevalent among infants and toddlers (Table 2). Retinoblastoma, which is rarely diagnosed in patients
older than 5 years, affected the youngest children in this study (mean age,
1.4 years); lymphoma and thyroid malignancies were most common among children
in the middle age group; and papillary thyroid carcinoma was found among the
oldest (mean age, 15.5 years).
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Table 2. Most Common Head and Neck Malignancies per Age Group
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The neck was the leading site of initial tumor involvement, particularly
the thyroid gland (Table 3). Other
sites included the orbit, skin (face and scalp), nasopharynx, and salivary
glands. Of note, no cases of pediatric cervical esophageal or parathyroid
malignancies were found in the SEER database.
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Table 3. Pediatric Head and Neck Tumor Sites
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INCIDENCE OF HEAD AND NECK CANCER
The incidence of all cancers diagnosed in children younger than 15 years
increased from 1973 to 1996 (Figure 2).
The average annual rate for cancers in this population (number of malignancies
per 100 000 person-years) rose 25% from 11.22 (95% CI, 10.70-11.74) for
1973-1975 to 14.03 (95% CI, 13.46-14.60) for 1994-1996 (Table 4). This rising trend also applied to those children younger
than 15 years with head and neck malignancies. Among malignancies of the head
and neck, the incidence rate increased 35% from 1.10 (95% CI, 0.94-1.26) to
1.49 (95% CI, 1.30-1.68) during the same period.
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Figure 2. The incidence of pediatric overall
and head and neck cancer from the Surveillance, Epidemiology, and End Results
tumor registry of the National Cancer Institute.
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Table 4. Comparison of Trends in Children Younger Than 15 Years: Head
and Neck vs Overall Cancer Incidence Rates*
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To determine whether these changes were also occurring in other regional
head and neck tumors with a known heritable component, we analyzed the 497
cases of retinoblastoma within the series. Retinoblastoma, which was the first
form of cancer directly linked to a gene, is caused by a deletion or mutation
of the q14 band of chromosome 13.16 After performing
an analysis identical to that performed in the other cohorts, we found that
the incidence of retinoblastoma had remained static from the 1973-1975 rate
of 0.36 (95% CI, 0.26-0.46) to the 1994-1996 rate of 0.36 (95% CI, 0.27-0.45)
despite significant rate increases at other head and neck sites (Table 4).
Rates for cancers demonstrated sex differences. The average annual rate
of malignancies among boys younger than 15 years rose significantly from 10.05
cases per 100 000 person-years for 1973-1975 to 15.11 cases for 1994-1996
(Table 4). During this same period,
the incidence of cancers among girls remained statistically unchanged. In
contrast, boys had slightly higher annual rates of head and neck malignancies
than girls in this age group; however, there was no statistically significant
difference over time. Overall, individual head and neck tumors that demonstrated
strong sex predilection were thyroid carcinoma (1:4 boy-girl ratio) and lymphoma
(5:3 boy-girl ratio) (Table 1).
COMMENT
This study represents the most extensive series of pediatric head and
neck malignancies to date and incorporates data from a very large, demographically
balanced tumor registry over an extended period. Malignancies of the head
and neck account for a significant proportion of all pediatric cancers. Leukemia
excluded, neural tumors and lymphoma have consistently been the leading types
of childhood cancer.2, 5, 7, 17-19
Overall, we were able to demonstrate incidence rates among all cases of pediatric
cancer that were consistent with those of other authors spanning similar periods.2, 17, 20 We acknowledge that
the observed increases among incidence rates may represent an artifact of
improved reporting over time—a potential variable inherent to any review
of a large population database. However, the reliability of the SEER database
is reflected in its role as the benchmark registry for cancer statistics in
the United States, and it remains the best available data source at this time.
The SEER data demonstrate that 12% of all children with cancer are affected
by a malignancy of the head and neck. This is higher than noted by Healy (5%)11 and lower than observed by Sutow (27%).9
Differences can be explained by varying tumor inclusion criteria. In keeping
with the clinical focus of this study, we chose to include tumors such as
retinoblastoma and lymphoma. While the treatment of these malignancies has
traditionally fallen to other medical specialties, the head and neck surgeon
is frequently involved with their initial evaluation and diagnosis. We believed
that limiting our investigation to cancer of a specific anatomic region rather
than certain tumor histologic characteristics was more clinically relevant
for the practitioner.
The increase in head and neck cancer incidence among children younger
than 15 years has outpaced the overall rise in malignancies for this age group.
Epidemiologists reason that inheritable types of cancer form a small portion
of childhood tumors.21 Our finding that the
incidence of retinoblastoma has remained constant compared with other head
and neck tumors is suggestive that nongenetic causes may explain this trend.
Well-established environmental carcinogens implicated in the development
of pediatric head and neck tumors include ionizing radiation, excessive solar
exposure, and certain chemotherapeutic agents.22
Other potential carcinogens are suspected but not proven. Environmental pollution,
parental exposure to toxins, electromagnetic fields, and even infectious agents
have been mentioned. Perinatal factors such as prematurity and low birth weight
have also been associated with an increased risk of childhood cancer.23 Unfortunately, the heterogeneous nature of pediatric
head and neck malignancies makes the search for clear etiologic factors difficult.
The incidence of childhood cancer clearly varies by sex. The overall
cancer rates among girls younger than 15 years have remained statistically
unchanged from 1973 through 1996, while those among boys of a similar age
have experienced a significant increase. Remarkably, rates among boys moved
from lower to higher than those among girls during this period. This trend
has been confirmed by several studies.5, 7-8,17
When older children are included, girls are more likely to have a head
and neck tumor than boys. This change most likely reflects the high number
of adolescent girls affected by thyroid carcinoma. Depending on patient age,
head and neck cancer has either no sex bias or a female predilection. This
is in contrast to the clear male predominance seen with pediatric malignancies
in general. These sex differences for head and neck tumors were not observed
in younger children (<15 years). Further research aimed at identifying
causes of the growing rate of childhood cancer may profit by exploring this
discrepancy.
Thyroid carcinoma represents a larger proportion of pediatric malignancies
than previously reported. Our finding that 21% of head and neck tumors were
thyroid carcinoma was higher than described by Jaffe and Jaffe (5%),10 Sutow (7%),9 and Cunningham
et al (10%).12 Indeed, papillary thyroid cancer
was the leading pathologic diagnosis in this study, which reinforces the need
for a thorough thyroid examination by the pediatrician when routinely evaluating
adolescent girls.
This study builds on previous work by quantifying the changing incidence
of pediatric head and neck cancer in a large population over time. The uncommon
occurrence of head and neck cancer in children had previously been an obstacle
to establishing the true incidence and ongoing trends of this disease. The
SEER database includes a large number of patients with an extended period
of follow-up. These features ameliorated the prior limitations of studying
head and neck cancer in children.
In conclusion, the incidence of head and neck malignancies in the United
States from 1973 through 1996 among children younger than 15 years increased
at a greater rate than childhood cancer in general. Reasons for this discrepancy
remain unclear, although research on a variety of potential carcinogens is
ongoing. Continued surveillance is necessary.
AUTHOR INFORMATION
Accepted for publication November 5, 2001.
This study was presented at the 16th Annual American Society of Pediatric
Otolaryngology Meeting, Scottsdale, Ariz, May 11, 2001.
We would like to thank Dennis H. Kraus, MD, Division of Otolaryngology–Head
and Neck Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, for
his critical review of the manuscript.
Corresponding author and reprints: James T. Albright, MD, Division
of Pediatric Otolaryngology–Head and Neck Surgery, Children's Hospital
and Health Center, 3030 Children's Way, Suite 402, San Diego, CA 92123 (e-mail: jtalbright{at}yahoo.com).
From the Department of Otolaryngology–Head and Neck Surgery (Dr
Albright) and the Division of Oncology Data Services (Mr Topham), Thomas Jefferson
University Hospital, Philadelphia, Pa; and the Department of Surgery, Alfred
I. DuPont Hospital for Children, Wilmington, Del (Dr Reilly). Dr Albright
is now with the Division of Pediatric Otolaryngology–Head and Neck Surgery,
Children's Hospital and Health Center, San Diego, Calif.
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