Childhood Cancer in the Primary Care Setting Mohamed Radhi, M.D. Associate Professor, UMKC Pediatric Hematology/Oncology/BMT Children s Mercy Hospital
I will discuss: Overview of childhood cancer Presentation signs and symptoms Epidemiology Diagnostic challenges Delay in diagnosis Final remarks
Overview of Childhood Cancer
Overview Childhood cancer is rare among childhood diseases. However, it is the second leading cause of death following accidents Childhood cancer is a challenging disease entity due to its rarity in nature, and it often presents with symptoms that overlap with more common, benign diseases such as ITP or rheumatologic disorders Childhood cancer can present at any age or in any organ. Although childhood cancer is usually a random event, it can be predisposed by certain genetic, familial, or immunologic disorders. Childhood cancer, can be a true life-threatening medical emergency that requires immediate and swift action to prevent loss of life.
Among the 12 major types, Leukemias and brain tumors account for more than half of all cases. The median age at diagnosis is six years old. Some forms of pediatric cancer have a 5 year survival rate of more than 90%, while others have a 5 year survival rate of less than 2%. In 80% of cases, a child s cancer diagnosis is delayed until the disease is very advanced and has spread to other parts of the body. As a stark comparison, this only occurs in 20% of adult cancer cases. Childhood cancers tend to be more aggressive than adult cancers, so this late diagnosis may significantly affect the 5- year survival probability of the child.
Presentation Table 1 Common childhood malignancies and associated clinical red flags that should prompt workup by a pediatric oncologist 7,8 Malignancy History/Symptom Sign Acute leukemia (ALL, AML) Fever Pallor Bone pain or limp Infection (particularly of an unusual location, severity, or organism) Anorexia, weight loss Fatigue Bruising Petechiae Mucosal bleeding Dyspnea Orthopnea Headache Double vision Fever Anemia WBC abnormalities (e.g. leukocytosis, peripheral blasts, leukopenia, neutropenia) Lymphadenopathy, generalized Hepatosplenomegaly Thrombocytopenia DIC Gingival hypertrophy Mediastinal mass Pleural effusion Cranial nerve palsy Lymphoma (NHL, HL) CNS tumors Lymph node swelling Fever Weight loss Night sweats Pruritis Bruising Petechiae Cough Dyspnea Orthopnea Pain Headache Vomiting Vision changes Irritability Weakness Loss of coordination Loss of developmental milestones Declining school performance Lymphadenopathy (nodes enlarged, firm, fixed, nontender) Fever Cytopenias (if bone marrow involvement) Hepatosplenomegaly Mediastinal mass Abdominal mass Intussusception Cranial nerve palsy Neurologic deficits (i.e. weakness, sensory loss) Ataxia Papilledema Parinaud s (i.e. paralysis of upward gaze) Full anterior fontanelle Abnormally increasing head circumference Seizures Neuroblastoma Wilms tumor Bone sarcoma (osteosarcoma, Ewing sarcoma) Soft tissue sarcoma (rhabdomyosarcoma and other rare sarcomas) Radhi et al Prim Care Clin Office Pract 42 (2015) 43 55 Enlarging abdomen Anorexia Weight loss Pallor Bruising Petechiae Bone pain Fever Irritability Diarrhea or constipation Back pain Enlarging abdomen Hematuria Constipation Abdominal pain Bone pain Deformity Swelling Anorexia, weight loss Symptoms due to mass effect (e.g. constipation from pelvic tumor) Palpable mass Deformity Swelling Pain Symptoms due to mass effect Abdominal mass Posterior chest mass Cytopenias Periorbital bruising ( raccoon eyes ) Proptosis Opsoclonus myoclonus ataxia syndrome Horner syndrome Bony masses Hypertension Weakness or paralysis Abdominal mass Hematuria, gross or microscopic Hypertension Palpable mass Deformity Swelling Pathologic fracture Pulmonary nodules Palpable mass Deformity Various, depending on location of tumor
Epidemiology
Epidemiology Every day in America, 43 children are diagnosed with cancer. Each year in the U.S. approx. 12-13 K children between the ages of birth and 19 years of age are diagnosed with cancer. This translates to about 1 in 7000 children diagnosed with cancer, and childhood cancer accounts for about 1% of all cancer diagnoses in the United States. There is no known cause of childhood cancer, and it occurs regularly and randomly across all ethnic groups. Some children are even born with cancer The 5-year survival rates for all childhood cancers combined increased from 58.1% from 1975 to 1977 to 79.6% from 1996 to 2003. Although the survival rate for childhood cancers has increased from less than 50% in 1970 to almost 80% today, cancers remain the leading cause of childhood death from disease.
Epidemiology In the 12400 new diagnoses/year < 20 yo 0-14 = 8,700 15-19 = 3,700 The probability of a newborn developing cancer by age 20 is between 0.3 0.32%, or ~ 1/300 1,500-1,600 deaths/year in children < 15 years of age
Age Specific Incidence Rates for Childhood Cancers
Most Common Childhood Cancers
Age-Adjusted and Age Specific Cancer Incidence SEER 2005-2009
Age Specific Incidence Rates for Childhood Cancers Most common cancer < 15 yrs leukemia > 15 yrs lymphoma Second most common < 10 yrs CNS tumors 10-14 yrs lymphoma > 15 yrs leukemia
SEER Delay-Adjusted Incidence and Mortality For ALL Childhood Cancers, 1975-2011
Trend in Survival Rates for Specific Childhood Cancers
Diagnostic Challenges
Diagnostic Challenges Childhood cancer can be difficult to diagnose in the primary care setting. For a pediatric oncologist, the index of suspicion of cancer is very high. The opposite for the general physician with Only about 1-2 percent of all cancers in western industrialized nations occur in children The presenting signs and symptoms are often nonspecific and mimic those of common childhood conditions such as viral infections or rheumatological disorders. There may also be a general reluctance to consider such a diagnosis as cancer carries with it the fear of death, is rare in childhood, and is an unfamiliar diagnosis for the non-specialist
Diagnosis Delay of Childhood Cancer Because pediatric cancers often present with vague symptoms, it is not surprising that there is often a delay in diagnosis. Appropriate early diagnosis and treatment require primary care physicians and parents to be aware of early symptoms of childhood malignancies. Tumors in children are very invasive but are more responsive to treatment than adult tumors. Misinterpretation of ambiguous cancer symptoms by patients, parents, and physicians may lead to diagnosis delay
Diagnosis Delays in Childhood Cancer Tam Dang-Tan, MSc, Eduardo L. Franco, DrPH
Diagnosis Delays in Childhood Cancer Tam Dang-Tan, MSc, Eduardo L. Franco, DrPH CANCER August 15, 2007 / Volume 110 / Number 4
Final Remarks Making the diagnosis of a childhood malignancy is often very difficult in the early stages of disease. Many of the presenting symptoms are common and typically not caused by cancer. Early diagnosis of childhood cancer is critical, as survival rates have increased dramatically over the past decades. Prolonged delay in diagnosis is common, especially for brain tumors and certain lymphomas (Hodgkin disease). When one encounters symptoms suspicious for a childhood malignancy, it is imperative that the child be referred to a pediatric cancer center.
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