Cancer
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Cancer in children differs from adult cancer in many ways. In adult cancer, there is often a strong environmental relationship. Children have not lived long enough for exposure to carcinogens and may not present for diagnosis until symptoms appear. By the time of diagnosis, 80% of cancers are metastatic. Cancer in children is treated aggressively with periods of exacerbations and remissions causing psychological distress for parents and families.
In children under 15 years of age, cancer is the second leading cause of death. However, cure rates have improved over the years and greater than 70% of children diagnosed with cancer will survive. As the number of survivors increases, so too does the late effects of cancer therapy, as well as secondary malignant neoplasms.
Cancer refers to a group of diseases in which there is abnormal cell growth. The neoplasm may be benign or malignant. Leukemia is the most common form of cancer in children, followed by brain tumors.
Childhood cancer is the second leading cause of death in children ages 1 to 14. Although survival rates and prognosis have improved over the years, it still continues to be a devastating illness. Leukemia continues to be the most frequent type of cancer, followed by brain tumors.
Cancer results when the body is unable to regulate cell production, causing altered cell differentiation and growth. Cell differentiation is defined as the process when dividing cells become more specialized, acquiring the structure and function of the cells they replace. Cell proliferation is defined as the process by which cells divide and reproduce. Cancer cells grow rapidly, spread widely, and do not follow the normal process of cell differentiation and cell proliferation. As a result, malignant cells do not look like the normal cells of the tissue from which they arose, and they do not grow at the rate they should.
An understanding of the cell cycle assists in understanding the process of malignancy as well as treatment options. Chemotherapeutic agents disrupt the cell cycle of proliferating malignant cells with as little damage to normal cells as possible. They are classified as noncell cycle specific or cell cycle specific.
Once diagnosed, many of the cancers are classified according to staging. Staging depends on the size of the main tumor, the presence or absence of metastasis, and the extent or spread to nearby lymph nodes. As the staging number increases, the prognosis becomes worse.
LEUKEMIA
Leukemia is the most common malignancy in children under the age of 15. It is a broad classification of malignant neoplasms of cells that arise from the hematopoietic stem cell. The two most common forms in children are acute lymphocytic leukemia (ALL) and acute myelogenous leukemia (AML). ALL accounts for 75% of all childhood leukemias. The peak incidence occurs in children at 4 years of age. Viruses, radiation, chemical and drug exposure, familial predisposition, and a variety of chromosomal aberrations have been cited as precipitating factors.
AML accounts for 15-20% of childhood leukemia and can be seen at any age. It is often associated with Down syndrome, exposure to radiation and chemotherapy from previous cancer, and exposure to toxins.
Pathophysiology
A leukemic cell is a type of immature white blood cell that accumulates in the marrow, blood, and tissue. Because they are immature and poorly differentiated, they have a prolonged life span and rapid rate of proliferation. Production of normal red cells, white cells, and platelets is inhibited. A lymphoblast is an immature white cell that crowds out normal cells, resulting in pancytopenia and immunosuppression. In a child with ALL, the bone marrow may be replaced by 80-100% blast cells.
Leukemic cells can cross the blood-brain barrier, causing involvement of the central nervous system (CNS). Because of this, presenting symptoms in ALL include fever, pallor, bruising, and bone pain. Lymphadenopathy, splenomegaly, and hepatomegaly caused by infiltration of lymphoblasts are common in ALL. In AML, children may present with vague flulike symptoms, or symptoms may be severe and life-threatening with bleeding and severe hemorrhage. There is a higher incidence of CNS involvement at the time of diagnosis.
A bone marrow aspiration and blood work confirm the diagnosis. In ALL, the white blood count and age of the child at diagnosis are prognostic indicators. Children between the ages of 3 and 5 and those with a white blood cell count less than 50,000/mm3 have the best prognosis. Children who are younger than 2 years or older than 10 years of age at diagnosis and those with a white blood cell count over 50,000/mm3 have a poorer prognosis.
Treatment
Chemotherapy is the treatment of choice and is started shortly after confirmation of diagnosis. The goal of the first phase is remission, meaning that there is no detectable sign of leukemia on physical exam or lab
results. This usually occurs within 4 weeks. CNS prophylaxis is also administered intrathecally into the cerebrospinal fluid space.
results. This usually occurs within 4 weeks. CNS prophylaxis is also administered intrathecally into the cerebrospinal fluid space.
Consolidation is the next phase of treatment and starts after remission has been attained to further decrease the number of leukemic cells in the body. Chemotherapy is again given in high doses to eradicate any residual leukemic cells. This phase is intense and lasts several months.
The goal of maintenance therapy is to prolong remission with monthly blood counts to evaluate the marrow’s response to the drugs. Almost 80% of children achieve a long-term disease-free life. The maintenance phase lasts for 2-3 years after diagnosis.
HODGKIN’S DISEASE
Hodgkin’s disease in one of two types of malignant lymphomas found in young adults. Its peak incidence occurs in the early twenties with a second peak incidence found in adults over the age of 50. In individuals diagnosed in their twenties, the incidence of men and women are equal, whereas for those individuals diagnosed in their fifties, there are more men than women diagnosed. While no known single agent has been reported as responsible for Hodgkin’s disease, several studies have identified possible inflammatory reaction to a viral infectious agent, possibly the Epstein-Barr virus.
Pathophysiology
Hodgkin’s disease most often originates in the cervical lymph node regions, and a definitive cure is possible depending on staging, histology, and time of diagnosis. In 60-90% of cases individuals present with nontender enlarged lymph nodes. In children, anorexia, malaise, and weight loss may also be accompanying symptoms. Hodgkin’s disease is classified as either “A” or “B” depending on whether or not significant weight loss, fever, or night sweats are present (“A,” not present; “B,” present). As the disease progresses metastasis to the liver, lungs, digestive tract, and spleen may occur.
Hodgkin’s disease most often originates in the cervical lymph node regions, and a definitive cure is possible depending on staging, histology, and time of diagnosis.