Child with Cancer Flashcards
Pediatric Cancer Definition
A group of chronic diseases characterized by uncontrolled growth and spread of abnormal cells which, if not adequately treated, results in the death of the patient
What is Cancer?
Genetic mutation: permanent DNA alteration. Can be inherited or caused by external toxins
Genetic Mutation results in:
- lack of differentiation
- loss of contact inhibition
- unregulated growth
- cellular immortality
Cancer is Caused by…
abnormal cellular growth, altered cell begins to multiply as directed by the altered genetic structure of its DNA and the absence or inactivation of tumor suppressor gene
Cancer starts as a genetic mutation (something goes wrong at the DNA level). A result of a newly acquired mutation at the DNA level, beginning in a single cell. DNA mutation results in lack of cell differentiation, loss of contact inhibition, unregulated growth, and cellular immortality. all of these cause the clinical manifestations of the disease.
Lack of cell differentiation
cells remain immature and fail to do work of normal cell
Loss of contact inhibition
normal cells stop dividing when they come in contact with other cells, but cancer cells crowd out normal cells
Unregulated growth
cancer cells multiply out of control - lack feedback mechanism that normal cells have. Mechanisms that tell the cell to die or stop dividing
Cellular immortality
failure of apoptosis/intrinsic suicide program
What Causes Childhood Cancer
Unknown (in most cases)
Not anything anyone did (or failed to do)
Related to peak growth
Possibly related to environmental agents (electromagnetic fields, chemicals, pesticides, viruses)
Kids cells are already growing fast and this might contribute
Certain cancers are related to peak growth periods, and there is an association between certain types of cancers occurring at certain ages
Oncogenes
activated through mutation of a proto-oncogene that normally maintains cellular growth & control; once activated to oncogene, uncontrolled cell growth (primary characteristic of cancer cells) results. Have been identified in leukemia, lymphoma, & some solid tumors.
Tumor-suppressor Genes
Arise from genes that normally suppress cancer formation but have lost their suppressor function, leading to uncontrolled growth. Have been identified in osteosarcoma, rhabdomyosarcoma, leukemia, retinoblastoma, and Willms tumor
Children at Increased Risk for Cancer (4)
Genetic predisposition (down syndrome: leukemia. Li-Fraumeni syndrome, retinoblastoma gene)
Immunodeficiency: higher risk because cancer has to do with immune system (congenital/acquired, treatment-related)
History of cancer
Prior cancer treatment
Classification of Childhood Cancer
Childhood cancer is not a single disease, but a wide range of diseases that are classified into two broad subcategories:
1. Hematological malignancies (leukemias and lymphomas)
2. Solid tumors
Within these classifications, tumors are named based on the type of tissue and/or site that the cancer develops in.
Three germ layers
Developing embryo has 3 germ cell layers:
- ectoderm (outermost layer - skin, mouth, nose, and anus)
- mesoderm (middle layer- blood vessels)
- endoderm (inner layer - respiratory, GI)
All body tissues arise from one of the embryonal tissue layers
Most childhood cancers arise from which embryonic germ layers
and adult?
Mesoderm and Ectoderm
(tissues deep within body)
Often referred to in pathology reports.
Don’t see skin & bowel cancers in children
And adult cancers arise from the endoderm (tissues exposed to the environment)
Ectoderm
CNS (brain, spine, meninges)
Mesoderm
blood
lymphatics
bone
muscle
pleura
heart
kidney
gonads
Endoderm
Epithelium
GI tract
Thyroid
Common Types of Childhood Cancer: Hematological
Leukemia (blood/marrow)
Lymphoma (lymph)
Common Types of Childhood Cancer: Solid Tumors (6)
CNS tumors (brain/spine)
Neuroblastoma (SNS)
Rhabdomyosarcoma (muscle)
Wilms tumor (kidney)
Ewing’s sarcoma, osteosarcoma (bone)
Retinoblastoma (eye)
Leukemia
“leuk” = white
“emia” = blood
Malignancy of blood-forming tissue
The blood has lost its red colour because it is full of white blood cells. Very low hemoglobin and platelets because of invading white blood cells
ALL
Acute Lymphoblastic Leukemia
AML
Acute myeloid (or myelogenous) leukemia
Leukemia what is it?
Malignant cell = “blast”
Blasts in leukemia:
- fail to mature
- crowd normal cells
Normally, the bone marrow is busy manufacturing precursors that will form healthy RBCs, WBC, and platelets. In leukemia, the malignant “blasts” fail to mature.They cannot do the job of normal white cells and they continue to divide, and crowd the bone marrow space, failing to allow production of normal cells.
Diagnosis usually occurs when blast cells move out into peripheral blood. Less ability of the blood to clot properly.
Bone Marrow Involvement Symptoms (HIGHLIGHTED)
Fever
Petechiae (blood coming into the interstitial spaces)
Fatigue
Types of Childhood Lymphoma
Hodgkin disease: Most common in adults and adolescence ages 15-19 (HIGHLIGHTED)
Non-Hodgkin lymphoma - can occur at any age (HIGHLIGHTED)
Lymphoma
a cancer of the lymphatic system, which is part of the body’s antigen-fighting network
includes the lymph nodes, spleen, tonsils, thymus gland and bond marrow. Lymphoma can affect all those areas as well as other organs throughout the body
Difference between HL and NHL
Those with Hodgkin’s lymphoma have large cancerous called Reed-Sternberg (RS) cells. Those with NHL do not have these cells
Symptoms of Lymphoma
Lymphoma may not always cause symptoms in its early stages. Instead, a doctor may discover enlarged lymph nodes during a physical examination. These may feel like small, soft nodules under the skin. A person may feel the lymph nodes in the:
- neck, upper chest, armpit, stomach, grooin
Common early symptoms:
- chills, cough, fatigue, enlarged spleen, fever, night sweats, itchy rash, SOB, skin itching, loss of appetite, stomach pain, unexplained weight loss
CNS Tumors
LOCATION!
Brains do not care about the cause of the injury, only the area of the brain which is injury. Location determines symptoms, gives indication as to what the tumor type is and can help predict prognosis
Location is important to what sympttoms we are going to see
CNS Tumors: Primary
arise from normal cells within the brain
name includes cell of origin +/- location
“benign” or malignant.
Benign: noncancerous but can be just as serious in some situations.
CNS Tumors: Metastatic
originate outside CNS
hematogenous spread
always malignant
symptoms -> primary diagnosis or relapse
Hard time getting treatment to brain because not all chemo drugs cross blood-brain barrier
Metastatic has poor prognosis
Tumor Grading: Benign
Typical of cell of origin
Decreased mitosis/necrosis
Slow growing/low grade
Localized
May be malignant by location
These tumors do not look like cancer under the microscope and histologically are typical of the cell of origin. They have minimal to no mitosis or necrosis, are considered slow growing or low grade. These tumors do not invade areas outside the site of origin but may be considered malignant by location. Chemo doesn’t work so well because it targets fast-growing cells
Tumor Grading: Malignant (brain cancer)
Greater differentiation
Increased mitosis/necrosis
Fast growing/high grade
Invade surrounding brain tissues
Metastasize outside CNS
These tumors under the microscope, look like cancer. They histologically have greater differentiation or anaplasia and mitosis or necrosis, which is consistent with cancer. They often do not resemble surrounding brain tissues, and may metastasize to other areas of CNS and in rare cases, to other areas of the body.
Neuroblastoma
Tumors arising from neural crest tissue:
- sympathetic nervous system
- adrenal gland
- diagnosis made after metastasis has occurred
Neuroblastoma occurs primarily in:
- infants/toddlers
- preschoolers
- rarely past early school age
Tissue of a Neuroblastoma
The cells of the tumor are primitive neuroblasts that differentiate into neuroblastoma
Very primitive tissue tissue. Usually originates in abd. area (base of spinal column or adrenal gland)
Slow-growing -> usually diagnosed in end-stage (very treatable if found early)
very rare
Solid tumors -> may present with abdominal swelling or pain 4 stages
The younger you are, the better you will do. If neuroblastoma found early (baby) tumor is removed & then no further chemo because baby fights cancer -> watch really closely. Sometimes is just goes away
5% long-term survival if found at age 3 years
Exclusively a pediatric cancer. None survive more than 10 years
