Kaplan Chapter 20 - Onc

Front 1

Acute Lymphoblastic Leukemia: Epi

Back 1

77% of all childhood leukemias

Front 2

Acute Lymphoblastic Leukemia: Initial presentation

Back 2

Nonspecific, brief onsent
*Fever, anorexia, fatigue
* Bone and joint pain (1/4), especially lower extremities

Front 3

Acute Lymphoblastic Leukemia: Later Signs/Sx

Back 3

1. Bone marrow failure: pallor, brusing, epistaxis
2. Petechiae, purpura, mucous membrane bleeding, LAD, HSM, joint swelling

Front 4

Acute Lymphoblastic Leukemia: Key to Bone/Joint Pain

Back 4

1/4 ALL patients present this way so have to keep this in the differential for bone/joint pain

Front 5

Acute Lymphoblastic Leukemia: LAD distribution

Back 5

- Suprclavicular and axillary are more suspicious

Front 6

Acute Lymphoblastic Leukemia: Dx Tests

Back 6

1. Peripheral Blood
2. BM aspirate

Front 7

Acute Lymphoblastic Leukemia: Peripheral blood findings

Back 7

a. anemia
b. thrombocytopenia
c. OFTEN do not see malignant cells
d. Most WBC < 10k --> Atypical lymphocytes

Front 8

Acute Lymphoblastic Leukemia: BM findings

Back 8

- Lymphoblasts

Front 9

Acute Lymphoblastic Leukemia: Tx

Back 9

1. Remission Induction
2. CNS Treatment (Phase 2)
3. Maintenance

Front 10

Acute Lymphoblastic Leukemia: Remission Rates

Back 10

98% in 4-5w

Front 11

Acute Lymphoblastic Leukemia: CNS Treatment

Back 11

Intrathecal and intensive systemic --> Decreases CNS relapse rate to 5%

For all ALL b/c ALL likes to hide in CNS (And testes)

Front 12

Acute Lymphoblastic Leukemia: Maintenance phase time

Back 12

2-3y
*Includes intrathecal therapy as well

Front 13

Acute Lymphoblastic Leukemia: What Tx NOT used

Back 13

*Radiation: Poor brain penetration, hard to irradiate all of blood...
*Many side effects

Front 14

Acute Lymphoblastic Leukemia: Complications

Back 14

1. Major impediment is relapse
2. Pneumocytis carinii
3. Other infections

Front 15

Acute Lymphoblastic Leukemia: Relapse Rates and Sites/Characteristics

Back 15

*15-20% overall
*Increased ICP or isolated CN palsies
* Testicular relapse in 1-2% of boys

Front 16

Acute Lymphoblastic Leukemia: Tumor Lysis Syndrome

Back 16

*No exclusive to this --> but any large tumor load (high WBC count > 50K or so

a. Hyperuricemia
b. electrolyte imbalance, K, P, Ca
c. Renal failure

Front 17

Acute Lymphoblastic Leukemia: Tumor Lysis syndrome Tx

Back 17

1. Hydration BEFORE and after
2. alkalinize urine
3. Prevent uric acid formation (pharm)

Front 18

Acute Lymphoblastic Leukemia: Prognosis

Back 18

VERY Good
1. > 80% 5-year

Front 19

Acute Lymphoblastic Leukemia: Bad prognostic factors

Back 19

1. Age < 1 or > 10y at Dx
2. > 100k WBC
3. Slow initial reponse
4. Chromosomal abnormalities

Front 20

Hodgkins Disease: Age Range

Back 20

Ages 15-19 (And another ~ 50y)

Front 21

Hodgkins Disease: Histo

Back 21

Reed-Sternberg Cell: Large cell w/ multilobulated nuclei

*Four subtypes

Front 22

Hodgkins Disease: Possible etiology

Back 22

EBV (But more associated with NHL)
Immunodeficiencies do predispose

Front 23

Hodgkins Disease: Presentation

Back 23

*Depends on tumor location
1. Painless, firm, cervical or supraclavicular nodes (#1)
2. Anterior mediastinal mass
3. Night sweats, fever, weight loss, lethargy, anorexia, pruritus (ITCHING)

Front 24

Hodgkins Disease: Dx

Back 24

BEST: Bx of affected node

Front 25

Hodgkins Disease: Staging Tests

Back 25

CXR (All patient to look for mets)
CBC
ESR, ferritin, Copper
CT Chest abdomen
Bone Marrow if III/IV

Front 26

Hodgkins Disease: Staging

Back 26

I: Single node/site
II: Two+ nodes but same side diaphragm
III: Both side diaphragm
IV: Diffuse

Front 27

Hodgkins Disease: Tx Determined by

Back 27

1. Disease stage, patient age
2. Size of mass
3. Hilar nodes

Front 28

Hodgkins Disease: Tx modalities

Back 28

Chemo
Radiation

Front 29

NHL: Origins

Back 29

*Malignant proliferation of lymphocytes: T, B, or intermediate origin

Front 30

NHL: Ages vs. Hodgkins

Back 30

- Younger

Front 31

NHL: Possible etiology

Back 31

EBC - Major role in Burkitts
1. 95% EBV genome in equatorial africa
2. Also congenital or acquired immunodeficiency predispose

Front 32

NHL: Subtypes

Back 32

1. Lymphoblastic -- Usually T
2. Small, noncleaved cell lymphoma -- B
3. Large Cell -- T, B, Intermediate

Front 33

NHL: Presentation

Back 33

*Depends on Locations
1. Anterior Mediastinal mass = respiratory Sx
2. Abdominal pain, mass, possible constipation

Front 34

NHL: Dx

Back 34

- Prompt b/c it's a very aggressive disease
1. Bx
2. Noninvasive: CT, CXR, Etc

Front 35

NHL: St. Jude Staging System

Back 35

I. Localized
II. Regional w/o mediastinal
III. Mediastinal/extensive
IV. Disseminated

Front 36

NHL: Tx

Back 36

1. Surgical excision of tumor if accessible
2. Chemo: To shrink
3. Radiation for some

Front 37

NHL: Cure Rate

Back 37

90% at 5-y for I and II

Front 38

Most common solid tumors in children

Back 38

Brain tumors (combined) -- 2nd most common cancers overall

Front 39

Brain Tumors: Mortality

Back 39

45% (Also highest morbidity)

Front 40

Brain Tumors: Age Range

Back 40

Most < 7y

Front 41

Brain Tumors: Sites

Back 41

2/3 are infratentorial

Front 42

Suspected Brain Tumors: Best initial Test

Back 42

Head CT

Front 43

Brain Tumors Location + Type: 0-1y

Back 43

Supratentorial
*Choroid Plexus + Teratomas

Front 44

Brain Tumors Location + Type: 2-10y

Back 44

Infratentorial
*Juvenile pilocytic astrocytoma
*Medulloblastoma (often supratentorial)

Front 45

Brain Tumors Location + Type: > 10y

Back 45

Supratentorial
*Diffuse astrocytoma

Front 46

Most common brain tumor histology overall:

Back 46

Astrocytoma

Front 47

Infratentorial Tumors: Most common and Outcomes

Back 47

*Astrocytoma most common --> Juvenile pilocytic most common sub-type
*Low grade; rarely invasive

Front 48

Classic site of Astrocytoma:

Back 48

Cerebellum

Front 49

Astrocytoma: Tx

Back 49

Surgery + Chemo/Rads

Front 50

Astrocytoma: Survival

Back 50

80-100% IF COMPLETE RESECTION

Front 51

Infratentorial Malignant Varieties:

Back 51

Malignant Astrocytoma (diffuse or fibrillary)
a. Anaplastic
b. Glioblastoma multiform --> Poor outcome

Front 52

Embryonal Brain tumors Include:

Back 52

Medullloblastoma (90%)

Front 53

Medulloblastoma: Ages

Back 53

Most or males 5-7y

Front 54

Medulloblastoma: Site

Back 54

Midline cerebellar vermis (most)

Front 55

Medulloblastoma: CT findings

Back 55

Solid, homengenous mass in postieor fossa

Front 56

Medulloblastoma: Obstructive Complications

Back 56

*Blocks 4th ventricle --> hydrocephalus --> Vomiting/ICP

Front 57

Medulloblastoma: Tx / Survival

Back 57

Chemo-Rads
*60-70% survival

Front 58

Third Most frequent tumor site?

Back 58

- Brainstem

Front 59

Brainstem tumors: Presentation

Back 59

- Motor weakness, CN deficits, cerebellar deficits (gait), signs of increased ICP

Front 60

Brainstem tumors: Varieties and outcomes

Back 60

- Low grade gliomas --> Surgery, OK outcome
- Diffuse intrinsic: Rads, palliative --> Very poor outcome

Front 61

Brainstem tumors: Survival if diffuse intrinsic

Back 61

Mean 12 mo

Front 62

Fourth most common Brain tumor: Name and % overall

Back 62

Ependymal Tumors (Ependymoma most common) - 10 % overall

Front 63

Ependymal Tumors: Site

Back 63

*From ependymal lining of ventricular system
*70% in posterior fossa --> 10% spread

Front 64

Ependymal Tumors: CT findings

Back 64

Well circumscribed, generally non-invasive

Front 65

Ependymal Tumors: Tx

Back 65

Surgery + Radiation (Chemo OK)

Front 66

Supratentorial Tumors: Key one to know? % of all?

Back 66

Craniopharyngioma --> 7-10% = most common supratentorial

Front 67

Craniopharyngioma: Site

Back 67

- Suprasellar region

Front 68

Craniopharyngioma: Aggressiveness

Back 68

Minimal

Front 69

Craniopharyngioma: Imaging Findings

Back 69

Calcification seen on X-Ray

Front 70

Craniopharyngioma: When to Think of?

Back 70

- Mass effect findings or --> Decreased growth and child endocrine deficits

Front 71

Craniopharyngioma: Morbidity

Back 71

Panhypopituitarism, growth failure, visual loss

Front 72

Craniopharyngioma: W/u and Tx

Back 72

- Endocrine studies
- Surgery and Rads --> NO CHEMO

*Hormone replacement after removal

Front 73

Eye Tumors: Most common

Back 73

Optic Nerve Gliomas:

*Most frequent tumor of the optic nerve

Front 74

Optic Nerve Gliomas: Histology

Back 74

Juvenile pilocytic astrocytoma

Front 75

Optic Nerve Gliomas: Course

Back 75

Benign, slowly progressive

Front 76

Optic Nerve Gliomas: Sx

Back 76

Unilateral vision loss + Proptosis + Eye deviation

*Optic atrophy
*Nystagmus
*Strabismus

Front 77

Optic Nerve Gliomas: Genetic Risk?

Back 77

- Neurofibromatosis

Front 78

Optic Nerve Gliomas: Tx

Back 78

*Observation initially

Chiasm: Radiation/Chemo
Surgery --> When confined or unsightly proptosis w/ visual loss or w/ visual deficits