Neoplastic Disorders

Question 1

Neoplastic Disorders

Answer 1

• Leading cause of death from disease in children past infancy
• Almost half of all childhood cancers involve blood or blood-forming organs

Question 2

Leukemia

Answer 2

• Most common form of childhood cancer
• Three to four cases per 100,000 Caucasian children younger than 15 years old
• More frequent in males over 1 year of age
• Peak onset is between 2 and 10 years of age

Question 3

Classification of Leukemia

Answer 3

• Leukemia describes a broad group of malignant diseases of the bone marrow and lymphatic system
• Complex disease with varying heterogeneity
• Classifications are increasingly complex

Question 4

Two forms of Leukemia

Answer 4

o Acute lymphoid leukemia (ALL)

o Acute nonlymphoid (myelogenous) leukemia (AML)

Question 5

Leukemia is an unrestricted ______________________ in the blood

Answer 5

proliferation of immature WBCs****

Question 6

Pathophysiology of Leukemia

Answer 6

• Leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body
• No “tumor” is present, but the same neoplastic properties are seen as in solid cancers
• Liver and spleen are the most severely affected organs
• Although leukemia is an overproduction of WBCs, the acute form often causes a low leukocyte count
• Immature cells do not attack and destroy the normal blood; destruction takes place by infiltration and subsequent competition for metabolic elements
• Depressed bone marrow function

Question 7

Consequences of Leukemia

Answer 7

• Depressed bone marrow function
  
  • Anemia from decreased RBCs
  • Infection from neutropenia
  • Bleeding tendencies from decreased platelet production
• Spleen, liver, and lymph glands show marked infiltration, enlargement, and fibrosis

Question 8

Diagnostic Evaluation of Leukemia

Answer 8

• Based on the history and physical manifestations - Tired, frequent illness- sore throat, fever, runny nose, bruising,
• Peripheral blood smear – blasts on smears
  
  • Immature leukocytes
  • Frequently, low blood counts
• Lumbar puncture to evaluate central nervous system (CNS) involvemen (To see if crossed the blood brain barrier)
• Bone marrow aspiration or biopsy

Question 9

Symptoms of Leukemia

Answer 9

Question 10

Managing Chemotherapeutic Agents

Answer 10

• Vesicants: Sclerosing agents even in minute amounts
• Interventions for extravasation
• Risk for anaphylaxis

Premedicate with zofran for nausea, get port or picc line placed
Before give benedril and Tylenol
VS – tachycardia, regular bp

Question 11

Managing Problems of Drug Toxicity (from chemo)

Answer 11

• Nausea and vomiting - Keep zofran everywhere
• Anorexia – make sure are eating
• Mucosal ulceration - Magic mouth wash (lidocaine (numbs), bacterial – ¯ swelling, mylanta – ¯acid)
• Neuropathy
• Hemorrhagic cystitis
• Alopecia
• Mood changes – from steroids, very sick and are hateful
• Moon face – from steroids

Question 12

Stages of leukemia treatment**

Answer 12

Induction, Consolidaiton, Maintenance, CNS prophylaxis

Question 13

Induction Stage**

Answer 13

• rapid, last 3-4 weeks, steroids, chemo IV
• Very fast! Dx then put on and start chemo the next day

Question 14

Consolidation**

Answer 14

• high dose methotrexate, length varies

Question 15

Maintenance**

Answer 15

eliminate all residual cells, 2-3 years

ALL tx is 3-4 years

Question 16

CNS prophylaxis**

Answer 16

given during all stages, intrathecal chemo

Question 17

If child with leukemia has fever…

Answer 17

go straight to ER and wear a mask at all times

Question 18

Lymphomas

Answer 18

Neoplastic disease originating in the lymphoid system; metastasizes to non-nodal sites

Hodgkin disease

Nonhodgkin’s lymphoma

Question 19

Hodgkin diseas More prevalent

Answer 19

in patients 15 to 19 years of age

Question 20

Non-Hodgkin lymphoma (NHL) More prevalent

Answer 20

in children younger than 14 years of age

Question 21

Classification of Hodgkin Disease

Answer 21

Classification A: Asymptomatic
Classification B: Temperature of 38°C (100.4°F) or higher for three consecutive days, night sweats, unexplained weight loss of 10% or more over the previous 6 months

Question 22

Hodgkin Disease

Answer 22

Neoplastic disease originating in the lymphoid system
Often metastasizes to the spleen, liver, bone marrow, lungs, and other tissues
Classified by histologic type

Question 23

CM of hodgkins disease

Answer 23

Asymptomatic enlarged cervical or supraclavicular lymphadenopathy is most common

· Knot over clavicle or in neck

Cough, abdominal pain, anorexia

· Phrenic nerve is compressed – what causes cough and abdominal pain

Question 24

Diagnosis of Hodgkin Disease

Answer 24

• CBC; T-cell and radiographic tests
• Lymph node biopsy for diagnosis and staging
• Bone marrow aspiration if the stage is advanced

Question 25

Lymph node biopsy will find presence of what type of cells for Hodgkins\*\*

Answer 25

Presence of Reed-Sternberg cells\*\*

Question 26

Stage I (Hodgkins)

Answer 26

one gropu of lymph nodes is affected

Question 27

stage II (Hodgkins)

Answer 27

two or more groups on the same side of the diaphragm are affected

Question 28

stage III (Hodgkins)

Answer 28

groups of lymph nodes above and below the diaphragm are affeted

Question 29

stage IV (Hodgkins)

Answer 29

Metastasis to organs such as liver, bones, or lungs

Question 30

stage A (Hodgkins)

Answer 30

absence of systemic symptoms at diagnosis

Question 31

stage B (Hodgkins)

Answer 31

systemic symptoms present at diagnosis (fever, night sweats, weight loss)

Question 32

Approximately 60% of pediatric lymphomas are

Answer 32

Non-Hodgkin Lymphoma (NHL)

Question 33

Clinical appearance of Non-Hodgkin Lymphoma (NHL)

Answer 33

Disease is usually diffuse rather than nodular Cell type is undifferentiated or poorly differentiated Dissemination occurs early, often, and rapidly Mediastinal involvement and invasion of meninges – goes to brain an spinal fluid

Question 34

Immunologic Deficiency Disorders

Answer 34

Human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) Horizontal transmission- sexual contact, blood or body fluids Perinatal or vertical transmission- Clinical Manifestations (don’t worry about) Lymphandenopathy Hepatosplenomegaly Oral candidasis Chronic or recurrent diarrhea FTT Developmental delay Parotitis – salivary glands

Question 35

Medulloblastoma-

Answer 35

cerebellum, fast growing, invasive, mets everywhere, poor prog

Question 36

Brain stem glioma-

Answer 36

aggressive, hard to resect, chemo resistant, affects cranial nerve function

Question 37

Ependymoma-

Answer 37

4th ventricle, causes hydrochephalus

Question 38

Astrocytoma-

Answer 38

slow, responds to chemo, low grade is good, high grade is bad

Question 39

Preopp Care

Answer 39

Watch for increased ICP Look at pupils for any deviations Administer dexamethasone - to decrease intracranial inflammation Stool softeners to prevent straining

Question 40

dexamethasone -

Answer 40

admin preopp to decrease intracranial inflammation

Question 41

Post opp care

Answer 41

Watch fluid administration – excess may cause/worsen cerebral edema Mannitol – decrease cerebral edema Pupils VS Watch for increased ICP

Question 42

Mannitol

Answer 42

admin postopp to decrease cerebral edema

Question 43

post opp position

Answer 43

Position on unaffected side, maintain head in alignment with remainder of body when changing positions · If brain tumor on R side then put pt on L side · Someone hold head while changing positions

Question 44

neuroblastoma

Answer 44

Most common extracranial tumor. Found in abdomen usually on adrenal gland Usually mets by time of diagnosis Staging determines treatment and prognosis

Question 45

Osteosarcoma

Answer 45

* Peaks in adolescents at peak of growth spurt (So pt old enough to know what is going on and know what want) * More common in males * Shrink tumor before surgery * Limb sparing or amputation - remove only affected portion of bone and replacing with endoprosthesis or cadaver bone * Include pt in planning

Question 46

Ewing Sarcoma

Answer 46

Highly malignant, rarer than osteo pelvis, chest wall, vertebra, midshaft long bones Prognosis determined by level of mets – mets very quickly

Question 47

Rhabdomyosarcoma

Answer 47

Soft tissue tumor Between 2-5 years old If in neck –may have compromised airway – devated trachea

Question 48

Most Common Sites of Rhabdomyosarcoma

Answer 48

Question 49

reproductive cancer

Answer 49

* Cervical- sexually active at young age, multiple partners - Typically from HPV * Testicular- rare in teens but dx 15-34 yoa * Teach about protection * Teach self exams

Question 50

Education

Answer 50

Much education for families and patients Must take into account developmental stage Must offer support emotionally Financial burdens Fear