The Care of a Child with Malignancy:
Malignancy: cells growing and spreading in a disorderly chaotic fashion
o S/Sx are dependent on where the cells are growing
Etiology
o Causes of most childhood cancers are unknown and for the most part they cannot
be prevented
o In rare cases children are at increased risk of developing a certain cancer (d/t
genetics) e.g. colorectal cancer
Incidence
o Approx. 11,600 new cases of pediatric cancer diagnosed in children ages 0-14
years in 2013
o Children account for approx. 2% of all cancer cases
o Major types: leukemia (disorder of WBCs) and brain and other CNS tumors,
which account for > half of new cases. Most common in white and Hispanic
children
o The overall incidence rate of invasive cancer in children has increased slightly
over the past 30 years
o Mortality rates for many childhood cancers declined by > 50%
o Combined 5-year survival for all childhood cancers is now 83% (up from 63% in
the 1970s) d/t improved treatment and high % of peds patients in clinical trials
Brain tumors: remain the leading cause of cancer-related death in children
CNS
o Controls our thoughts, memories, reactions, speech, understanding, and
temperament
o Our senses (vision, touch, hearing, smell, and taste) are all processed and
interpreted by the brain
o Our movements (balance and coordination) are orchestrated by the brain as well
o The functions for simple existence, such as breathing, heartbeat and BP are all
under the direction of our CNS
o Brain tumors can cause significant interference
Childhood Cancers
o Leukemia is most common
o Most common solid tumors of the CNS
are neuroblastoma and brain tumors
o Neuroblastoma: neuroendocrine tumors
which arrives at any neural crest
element within the CNS
Adrenal glands
Nerve tissue
o Renal tumors are also prevalent
Ex. Wilms tumor
, o Soft tissue
Ex. Rhabdomyosarcomas (muscle malignant cancer)
o Cancers of the eye
Retinoblastoma
o Bone
Osteosarcoma
Germ cells: fetal cells of the reproductive tissue (ovarian)
Sarcoma: malignant soft tissue or bone cancer
Common Presenting S/Sx of Malignancy
o Fever in the absence of illness
o Pain at the localizing site of the cancer
o Fatigue out of proportion to activities
o Headaches
o Lymphadenopathy
Supraclavicular lymph nodes
o Pallor
o Purpura
o Weight loss
o Neurological changes
o Mass of swelling
Leukemia
o Most common childhood cancer
o Disease involving the blood-forming tissues of the bone marrow, spleen, and
lymph nodes
o Specifically, it is the distorted and uncontrolled proliferation of WBCs
Can make less room for other blood cells (RBCs)
Anemia and thrombocytopenia
Hematopoiesis
o Formation and development of the various blood cells (RBCs, leukocytes, and
platelets) from the undifferentiated, pluripotent stem cell
o Process includes proliferation, differentiation, and maturation of cells into 2
distinct cell lines: lymphoid and myeloid
Lymphoid cells: B cells and T cells
Myeloid cells: RBCs, monocytes, granulocytes, and platelets
, Blood Cell Maturation
Leukemia
o In leukemia, normal
hematopoiesis is
altered, resulting in
arrested development
o Malignant stem cells
result in cells that
have lost their ability
to regulate cell
division and to
differentiate into
mature functional
cells
o Immature cells
accumulate in the marrow spaces, peripheral blood, and selected organs (spleen
and lymph nodes)
Splenomegaly: hard, nodular spleen
o Because the abnormally proliferating cells are so immature, they may be
identified at the immature, or “blast” or “stem” cell stage
o Production of normal hemopoietic cells is limited by the lack of space in the bone
o Symptoms due to a decreased number of mature, functional WBCs, platelets, and
RBCs.
Anemia and thrombocytopenia
o Classifications
The classification of the different leukemias is based on the predominantly
affected cell line and the level of cell differentiation
Major classifications
Acute lymphocytic leukemia (ALL) 75-80%
Acute myelogenous leukemia (AML) 20-25%
o Acute Lymphocytic Leukemia
2500 cases per year in the US
Peak age of onset: 4 years (preschool)
Malignant cell line involved is the immature lymphocyte, the lymphoblast
o Acute Myelogenous Leukemia
Frequency increases in late adolescence (teens)
Approx. 10% of childhood AML occurs in infants under 2 years of age
Most common type of leukemia in adults
Accounts for 30-50% of deaths from childhood leukemia (must more
serious in the peds population)
, o Clinical Presentation of Leukemias
Anemia: malaise, fatigue, pallor
Thrombocytopenia: gingival, cutaneous, or nasal bleeding
Neutropenia: fever, recurrent infections
Hepatosplenomegaly, abdominal pain, N & V
Bone pain (23%)
Lymphadenopathy
Anorexia, weight loss
CNS involvement (metastasis to the CNS)
CNS disease (>10% of cases at diagnosis): increased ICP,
headache vomiting, and visual disturbances
o Clinical Presentation of AML
May present with life-threatening sepsis or hemorrhage
Gingival hypertrophy
Extramedullary infiltration (i.e. into the soft tissues and skin)
Chloromas (myeloid sarcoma)
Leukemia cutis (infiltration of leukemia cells under the skin;
approx. 3%--colorless or slightly purple, “blueberry muffin”
appearance
o Work-up (AML)
Diagnostic procedures
Laboratory values
Bone marrow aspirate and/or biopsy
Biopsy
Staging
Imaging
o CT scans
o PET scan
o Bone scan
o MIBG scan
Metaiodobenzylguanidine (MIBG) is an agent that
is similar to adrenaline and localizes in certain types
of tumors in the body called neuroendocrine tumors.
MIBG is labeled with a small amount of radioactive
material, and pictures can then be made of the
distribution of this material after it is injected IV.
For this procedure, the body is exposed to a small
amount of radiation
It is used when your doctor suspects or has proof of
a neuroendocrine tumor. The scan will determine if
your tumor accumulates this agent, and if it does,
the distribution of tumor in your body
Diagnostic lumbar punctures
Malignancy: cells growing and spreading in a disorderly chaotic fashion
o S/Sx are dependent on where the cells are growing
Etiology
o Causes of most childhood cancers are unknown and for the most part they cannot
be prevented
o In rare cases children are at increased risk of developing a certain cancer (d/t
genetics) e.g. colorectal cancer
Incidence
o Approx. 11,600 new cases of pediatric cancer diagnosed in children ages 0-14
years in 2013
o Children account for approx. 2% of all cancer cases
o Major types: leukemia (disorder of WBCs) and brain and other CNS tumors,
which account for > half of new cases. Most common in white and Hispanic
children
o The overall incidence rate of invasive cancer in children has increased slightly
over the past 30 years
o Mortality rates for many childhood cancers declined by > 50%
o Combined 5-year survival for all childhood cancers is now 83% (up from 63% in
the 1970s) d/t improved treatment and high % of peds patients in clinical trials
Brain tumors: remain the leading cause of cancer-related death in children
CNS
o Controls our thoughts, memories, reactions, speech, understanding, and
temperament
o Our senses (vision, touch, hearing, smell, and taste) are all processed and
interpreted by the brain
o Our movements (balance and coordination) are orchestrated by the brain as well
o The functions for simple existence, such as breathing, heartbeat and BP are all
under the direction of our CNS
o Brain tumors can cause significant interference
Childhood Cancers
o Leukemia is most common
o Most common solid tumors of the CNS
are neuroblastoma and brain tumors
o Neuroblastoma: neuroendocrine tumors
which arrives at any neural crest
element within the CNS
Adrenal glands
Nerve tissue
o Renal tumors are also prevalent
Ex. Wilms tumor
, o Soft tissue
Ex. Rhabdomyosarcomas (muscle malignant cancer)
o Cancers of the eye
Retinoblastoma
o Bone
Osteosarcoma
Germ cells: fetal cells of the reproductive tissue (ovarian)
Sarcoma: malignant soft tissue or bone cancer
Common Presenting S/Sx of Malignancy
o Fever in the absence of illness
o Pain at the localizing site of the cancer
o Fatigue out of proportion to activities
o Headaches
o Lymphadenopathy
Supraclavicular lymph nodes
o Pallor
o Purpura
o Weight loss
o Neurological changes
o Mass of swelling
Leukemia
o Most common childhood cancer
o Disease involving the blood-forming tissues of the bone marrow, spleen, and
lymph nodes
o Specifically, it is the distorted and uncontrolled proliferation of WBCs
Can make less room for other blood cells (RBCs)
Anemia and thrombocytopenia
Hematopoiesis
o Formation and development of the various blood cells (RBCs, leukocytes, and
platelets) from the undifferentiated, pluripotent stem cell
o Process includes proliferation, differentiation, and maturation of cells into 2
distinct cell lines: lymphoid and myeloid
Lymphoid cells: B cells and T cells
Myeloid cells: RBCs, monocytes, granulocytes, and platelets
, Blood Cell Maturation
Leukemia
o In leukemia, normal
hematopoiesis is
altered, resulting in
arrested development
o Malignant stem cells
result in cells that
have lost their ability
to regulate cell
division and to
differentiate into
mature functional
cells
o Immature cells
accumulate in the marrow spaces, peripheral blood, and selected organs (spleen
and lymph nodes)
Splenomegaly: hard, nodular spleen
o Because the abnormally proliferating cells are so immature, they may be
identified at the immature, or “blast” or “stem” cell stage
o Production of normal hemopoietic cells is limited by the lack of space in the bone
o Symptoms due to a decreased number of mature, functional WBCs, platelets, and
RBCs.
Anemia and thrombocytopenia
o Classifications
The classification of the different leukemias is based on the predominantly
affected cell line and the level of cell differentiation
Major classifications
Acute lymphocytic leukemia (ALL) 75-80%
Acute myelogenous leukemia (AML) 20-25%
o Acute Lymphocytic Leukemia
2500 cases per year in the US
Peak age of onset: 4 years (preschool)
Malignant cell line involved is the immature lymphocyte, the lymphoblast
o Acute Myelogenous Leukemia
Frequency increases in late adolescence (teens)
Approx. 10% of childhood AML occurs in infants under 2 years of age
Most common type of leukemia in adults
Accounts for 30-50% of deaths from childhood leukemia (must more
serious in the peds population)
, o Clinical Presentation of Leukemias
Anemia: malaise, fatigue, pallor
Thrombocytopenia: gingival, cutaneous, or nasal bleeding
Neutropenia: fever, recurrent infections
Hepatosplenomegaly, abdominal pain, N & V
Bone pain (23%)
Lymphadenopathy
Anorexia, weight loss
CNS involvement (metastasis to the CNS)
CNS disease (>10% of cases at diagnosis): increased ICP,
headache vomiting, and visual disturbances
o Clinical Presentation of AML
May present with life-threatening sepsis or hemorrhage
Gingival hypertrophy
Extramedullary infiltration (i.e. into the soft tissues and skin)
Chloromas (myeloid sarcoma)
Leukemia cutis (infiltration of leukemia cells under the skin;
approx. 3%--colorless or slightly purple, “blueberry muffin”
appearance
o Work-up (AML)
Diagnostic procedures
Laboratory values
Bone marrow aspirate and/or biopsy
Biopsy
Staging
Imaging
o CT scans
o PET scan
o Bone scan
o MIBG scan
Metaiodobenzylguanidine (MIBG) is an agent that
is similar to adrenaline and localizes in certain types
of tumors in the body called neuroendocrine tumors.
MIBG is labeled with a small amount of radioactive
material, and pictures can then be made of the
distribution of this material after it is injected IV.
For this procedure, the body is exposed to a small
amount of radiation
It is used when your doctor suspects or has proof of
a neuroendocrine tumor. The scan will determine if
your tumor accumulates this agent, and if it does,
the distribution of tumor in your body
Diagnostic lumbar punctures