Cancers

Question 1

Causes of cancer

Answer 1

(1) Genetics
(2) Failure of immune system to distinguish between normal and abnormal cells
(3) Carcinogens

Question 2

Incidence

Answer 2

• About 16K new cases of pediatric cancer diagnosed in children 0 - 19
• White children highest incidence
• 5 year survival rate for all childhood cancers is 80%
• 10 year survival rate is almost 75%

Question 3

Most common childhood cancers

Answer 3

(1) Leukemia
(2) Brain & Spinal tumors
(3) Lymphoma

Question 4

Chemotherapy

Answer 4

(1) Antineoplastic drugs (to destroy abnormal tissue / get several different drugs - so there isn’t a resistance developed to that chemotherapy) –> Since tumors possess ability to develop resistance to chemo, a variety of drugs are used
(2) Pretreatment evaluation –> physiological preparation & psychological preparation. Chemo causes organ specific damage, for instance: the kidneys (are always blasted)… Before giving Adriamycin check for any heart problems
(3) Psychological preparation: ALL treatment is 2 to 3 years. Families need to be prepared for that
(4) Route: Oral, IM, IV, subcutaneous, intrathecal (into the spine)

Question 5

When do the side effects of chemotherapy peak?

Answer 5

Side effects peak at 7 - 10 days

Question 6

Side Effects of Chemotherapy

Answer 6

(1) Non-selectively kills rapidly dividing cells
(2) Bone marrow suppression - neutropenia (a decrease in neutrophils & platelets), anemia & thrombocytopenia (deficiency in platelets, takes longer for blood to clot)
(3) Gastrointestinal - N/V, mucosal ulcerations, esophagitis & constipation
(4) Alopecia
(5) Organ damage - specific to certain chemotherapy

Question 7

How do doctors’ connect “central line” to RA?

Answer 7

With either a BrovIac OR Portacath –> NEVER THRU PERIPHERAL VEINS (cannot handle chemotherapy)

Question 8

Bone Marrow & Stem Cell Transplant

Answer 8

Two Types: (1) Autologous (from self) / (2) Allogenic (from donor)

Question 9

Preparation for Allogenic Transplant…

Answer 9

Try to suppress the immune system so there isn’t graft vs. host

Question 10

Preparation for Autologous Transplant…

Answer 10

Try to wipe out whatever residual cancer is there before they harvest stem cells

Question 11

Preparation for Treatment

Answer 11

Conditioning: Before transplant happens, they have what is called a “conditioning phase” where they wallop child with chemo and radiation. Then receives bone marrow or stem cells by IV infusion.

Question 12

Engrafting

Answer 12

Usually occurs 14 - 28 days after transplant. If engraphment is going well, you will start to see an increase in WBC, RBC, and platelets

Question 13

Graft vs. Host

Answer 13

Donor’s WBCs begin to attack patients’ - MUST be biopsied to make sure. S&S of Graft vs. Host are:

(1) Rash
(2) Vomiting
(3) Diarrhea
(4) Fever
(5) Altered Liver Enzymes

Question 14

Biotherapy

Answer 14

• Using part of the human body to destroy cells and applies them to cancer cells (tumor specific antibodies)
• Group of drugs that stimulate body’s own immune response (Inteferon: signaling proteins that say “I am infected”)
• Vaccines (HPV) / Helping to prevent cervical cancer
• Molecular targeting (interference with metabolic pathways through enzyme destruction)
• Gene therapy (replace faulty gene with normal cell)

Question 15

Absolute Neutrophil Count (ANC)

Answer 15

Multiply # of WBC by the total % of neutrophils
(“polys” or “sags” are MATURE neutrophils / “bands” are IMMATURE)
*Normal ANC are in the 1,000s
Kids need 500 to go back to school

Question 16

Tumor Lysis Syndrome

Answer 16

• Lysis (explosion) of tumor cells and rapid release of contents of tumor cells into the blood stream.
• Can result from cancer itself (leukemia) or side effect of treatment.
• High levels of uric acid, K+, and phosphate
• Can cause cardiac arrhythmias and renal failure
• ALL and Non-Hodgkin’s Lymphoma
• Have to correct this before child gets chemo
• Allopurnol (IV) reduces levels of uric acid
• Bicarb (IV) lowers K+ (potassium) as well as phosphate

Question 17

Leukemia

Answer 17

• Most common cause of childhood cancer
• Proliferation of immature WBCs in blood forming tissues of body
• More common in males than females
• Peak at 2 - 6 years of age
  Long term disease free survival rates of ALL is 80%

Question 18

Leukemia - Pathophysiology

Answer 18

Alteration in the genetic makeup of WBC
Prevents maturation of WBC
**Immature WBCs replicate very quickly, and crowd out platelets and RBCs –> Lack bio-feedback mechanism

Question 19

Leukemia - Pathophysiology (cont)

Answer 19

• Replicates quickly forming immature or blast cells in bone marrow, crowding cells (WBCs, RBCs, and platelets)
• Replicate in great #s and are released into circulation
• Blast cells take over marrow. RBC & platelet cell production is altered –> anemia, thrombocytopenia
• Cells compete… and normal cells are deprived of essential nutrients of metabolism
• Infiltration of bone marrow causes: anemia, infection, bleeding, peticia (below nipples), fractures and bone pain - causes the expansion of the bone
• Cells infiltrate extra medullary sites (CNS, Liver, Spleen, Testies, Lymph Nodes)
• Classified as type of cell involved: T, B, early pre-B, or pre-B

Question 20

Does child have ALL?

Answer 20

Check for % of “blasts” in bone marrow (“blasts” are immature). If there are 30% or more WBCs that are blasts - indicates child has ALL

Question 21

Favorable Prognostic Factors for ALL

Answer 21

(1) Leukocyte count of less than 50,000
(2) Older than 2, less than 10
(3) CALLA positive
(4) Pre-B cell
(5) Female

Question 22

Nursing a child with leukymia

Answer 22

(1) Do not give them any live vaccines, vircella (chicken pox) or MMR
(2) Never take rectal temps
(3) Make sure they are really, REALLY well hydrated

Question 23

Brain Tumors

Answer 23

Most common: solid tumor
Confined to brain & spine
Classified according to cell histology & rate of tumor proliferation

Question 24

Hallmark Symptoms (Brain Tumor)

Answer 24

• Headache with morning vomiting related to child rising

- Ataxia, Visual disturbances, Fatigue, Loss of milestones, Poor school performance

Question 25

Brain Tumor (Management)

Answer 25

CT scan or MRI Surgery Chemotherapy Radiation - avoided in children less than 3

Question 26

Brain Tumor (Post Op)

Answer 26

(1) Vital Signs - fever post op could be due to stimulation of hypothalamus. If it persists for longer than 48 hours, then start to think about infection (2) Neuro - FIRST sign something is wrong is a change in the patient's LOC

Question 27

Brain Tumor (Medications)

Answer 27

(1) Opioids - but need to make sure you are not masking their LOC (2) Manitol - osmotic diuretic (3) Dextametadone - steroid to decrease inflammation (4) Antibiotics

Question 28

Neuroblastoma

Answer 28

- Most common malignant extra-cranial solid tumor of childhood - Occur in 1 / 10,000 live births - 1/2 of all cases under 2 (2+ already have metastasis) - Under 12 months, may experience spontaneous remission - Males > Females - More common in whites - 80% present with metastasis with symptoms in non-primary sites.

Question 29

Neuroblastoma (Pathophysiology)

Answer 29

(1) Originate from neural crest cells which normally develop into sympathetic nervous system and adrenal medulla (2) Most common sites: Adrenal gland (on top of kidneys) and Retroperitoneal Sympathetic Chain (bottom of lower back)

Question 30

Neuroblastoma (Clinical Manifestations)

Answer 30

65% have abdominal mass & protuberant firm abdomen Impaired ROM & mobility Pain & Limping Impaired bladder & bowel functions Chest: cough & respiratory compromise Neck: facial, periorbital edema & bruising Spinal Chord: inability to walk Fever & Hypertension

Question 31

Cause of Neuroblastoma

Answer 31

Chromosome #1 | Amplification of oncogene called MYCN, which causes uncontrolled cell growth

Question 32

How do we diagnose Neuroblastoma?

Answer 32

``` Chest X-Ray CT of chest, abdomen, and pelvis Bone marrow aspirations & IVP Skeletal survey Tumor samples ** Urinary excretions of catecholamines. VMA as well as HVA will be in urine ```

Question 33

Hodgkins Disease

Answer 33

Most common in adolescence and young adult (15) Originates in lymphoid system Can spread from lymph nodes to non-nodular sites, including: liver, spleen, bone marrow, LUNGS)

Question 34

Hodgkins Disease - Staging

Answer 34

Dependent on: (1) # of lymph node sites (2) extra nodular disease (3) history of symptoms Staged 1 - 4

Question 35

Hodgkins Disease - Clinical Manifestations

Answer 35

- Asymptomatic, enlarged cervical or supra-clavicular lymphadenopathy (NON TENDER) - Persistant non-productive cough - Abdominal pain - Low grade, intermittent fever - Weigh loss, night sweats (if stage B) - Nausea - Pruritus - itching

Question 36

How do you diagnose Hodgkins Disease?

Answer 36

Biopsy of lymph nodes: Sternberg-Reed cells ("owl eyes") CT scan & Gallium scan X-ray with chest, abdomen, & pelvis Bone Marrow

Question 37

Hodgkins Disease - Survival?

Answer 37

**Chemo, radiation either alone or combined. Nothing to surgically remove except sometimes the spleen Stages I & II --> 90% Stage III --> 80% Stage IV --> 70%

Question 38

Osteogenic Sarcoma

Answer 38

``` Most common bone cancer Peak at 10 - 25 years of age Primary sites: more than 1/2 in femur 9% of all childhood cancers In about 20% disease has spread by time kid is diagnosed ```

Question 39

Osteogenic Sarcoma - Clinical Manifestations

Answer 39

(1) Progressive, insidious, or intermittent pain at tumor site relieved by position change (flexion makes it feel better!) (2) Palpable mass (3) Limited ROM (4) Pathologic fracture at tumor site

Question 40

How to diagnose Osteogenic Sarcoma?

Answer 40

``` Rule out other causes History regarding the pain CT scan or MRI Bone Scan Needle or surgical bone biopsy **CBC serum Alkaline Phosphate (ALP) & Lactase Dehydrogenase (LDH). The higher these #s, the POORER the patient responds to treatment ```

Question 41

Osteogenic Sarcoma - Management & Prognosis

Answer 41

Surgery & Chemo | UNRESPONSIVE to radiation