Look for red flags that raise index of suspicion for childhood cancer
Roger L.Berkow, M.D., FAAP
Focus on Subspecialties
Each year, approximately 16,000 U.S. children between birth and age 19 are diagnosed
with cancer, and more than 40,000 children undergo cancer treatment. Cancer remains
the leading cause of death from disease in childhood.
The signs and symptoms of childhood cancer are nonspecific, and initial recognition
often is difficult. Paying attention to clinical red flags increases the index of
suspicion for childhood cancers.
September is Childhood Cancer Awareness Month, providing an opportunity to reinforce
the importance of early diagnosis. Following is a review of the epidemiology of childhood
cancer, important signs and symptoms that should raise concern for cancer, and guidance
for primary care pediatricians in the recognition and diagnosis of common pediatric
malignancies. Finally, suggestions are offered on how to support patients and families
in the event of a possible cancer diagnosis.
Epidemiology of childhood cancer
Cancer in children can occur at any age, with peaks of incidence during infancy when
neuroblastoma is most common and between the ages of 2 and 4 when leukemia is the
most common. The incidence drops through the school-age years and then begins to increase
Leukemia/lymphoma and cancers of the central nervous system represent the most frequent
cancers in childhood. The frequency of other childhood cancers is shown in the figure.
Signs and symptoms of childhood cancer are nonspecific and include many findings observed
in a variety of childhood disorders. These include fever, musculoskeletal symptoms,
pain, fatigue, pallor, bruising, bleeding, headaches, lymphadenopathy, and loss of
appetite, vomiting and weight loss.
Any symptom in isolation is less likely to be associated with a childhood cancer diagnosis
than if multiple symptoms are present or if the symptoms have persisted over time
and resulted in multiple medical visits.
Some symptoms considered red flags are more specific but still not diagnostic of malignancy.
Red flags include lymphadenopathy of greater than 2 centimeters (particularly if firm,
non-tender, fixed or in the supraclavicular area), morning vomiting (especially if
associated with headache or other neurologic symptoms), rapid vision changes with
or without papilledema, painless swelling, fever associated with pallor or persistent
fevers associated with night sweats.
Abdominal, bone or soft tissue masses, scrotal masses that do not trans-illuminate,
and urinary retention especially if associated with lower extremity weakness or flaccidity
are other red flag symptoms that should raise a high index of suspicion for a malignancy.
Lab, imaging studies
Initial screening laboratory studies for suspected childhood cancer include a complete
blood count with differential and reticulocyte count to assess for cytopenias or bone
marrow production problems; a comprehensive metabolic panel (or electrolytes, renal
and hepatic function) as well as a lactate dehydrogenase and uric acid levels (nonspecific
measures of increased cell turnover). Other laboratory studies can be tailored to
the specific disorder in question and should be done in the referral center.
Radiographic studies most frequently will begin with plain chest X-rays to evaluate
for mediastinal adenopathy or pulmonary metastasis or X-rays of a painful or affected
extremity or spine to evaluate for bony destruction. More specific radiography may
include an ultrasound of the abdomen or scrotum for initial evaluation of a suspected
Computerized tomography and/or magnetic resonance imaging for more definitive assessment
are best performed in the referral center where oncology-specific protocols are followed.
Indeed, it often is preferable for primary care providers to do less testing and/or
imaging and to consult an oncologist early about concerns and make a mutually agreeable
decision about how to proceed.
Supporting patients and families
The suspected diagnosis of cancer in a child causes significant anxiety for the patient,
the family and the pediatrician. It is critical to speak to the parent, and patient
if age appropriate, honestly about your concerns. Use the words cancer, tumor and
leukemia as appropriate; avoid vague expressions of “bad cells” or “mass.”
When referring to an oncologist for initial consultation, reassure the family that
you still are their child’s pediatrician and that you will remain involved throughout
Dr. Berkow is a member of the AAP Section on Hematology/Oncology.