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Flashcards in Ped Oncology Deck (69):
1

number one cause of disease related death in children?

cancer bro

2

*Most common cancers of childhood?

1) leukemia
2) CNS
3) soft tissue
4) non hodgkin and kidney
5) bone
6) hodgkin

3

is incidence of childhood cancer increasing?

Yes a little bit for the common tumors but not so much for the less common!

4

5 yr survival for common bone marrow related cancers

lowest to highest:
-AML
-NB
-ALL
-Wilms

5

Epidemiology of childhood cancer

-1/330 Americans develops cancer before 20

~46 U.S. children & adolescents diagnosed each day

6

Long-term effects of childhood cancer

-1:750 20-year-olds alive U.S. is a survivor of childhood cancer
-3/5 children diagnosed w/ cancer suffer long-term or late onset side effects

7

2 childhood cancers that may be increasing in incidence

1. ALL
2. Brain & CNS

8

Most improved outcomes has been for which childhood cancer?

ALL

9

Where are children usually treated for cancer?

>90% seen & treated at a COG affiliated institute

-Consistent tx across U.S; Don't need to travel somewhere else to get the best treatment

10

General points about causes of childhood cancers

Largely unknown:
-Specific chromosomal/genetic abnormalities, & ionizing radiation exposures explain only a small % of cases
-Environmental causes of childhood cancer have long been suspected by many scientists but have been difficult to pin down

11

Familial/genetic diseases associated with increased cancer risk:

1. Neurofibromatosis
2. Familial polyposis
3. Li-Fraumeni syndrome

12

Major categories of diseases linked with an increased cancer risk include:

1. Immune deficiencies
2. Metabolic disorders
3. Disorders of chromosome stability

13

*General points about presentation of childhood cancers

1. S/S of cancer are relatively non-specific and mimic a variety of more common childhood problems
2. Oncologists are highly suspicious for cancer; Primary care physicians are opposite
3. You have to think about the possibility of cancer before you can make the diagnosis

14

*Red flags for Leukemia

1. Unexplained fever(s) >101F >7 days
2. Petechiae
3. Unexplained anemia / pallor
4. Generalized lymphadenopathy
5. Hepatosplenomegaly
**6. Bone/joint pain (30%) not relieved w/ pain medications or that wakes from sleep (Expanding marrow space)
7. sudden weight loss
8. hypertension

15

*Red flags for Brain Tumors

Pediatric tumors are often situated that they interfere w/ CSF circulation-> in ↑ intracranial P. ->
-***Headaches & vomiting*** (early am especially) are common presenting signs

16

*Conditions Suggesting Radiographic Evaluation in Headaches

1. Presence of neurologic abnormality
2. Ocular findings, papilledema
3. Persistent vomiting/ increasing or Preceded by recurrent HAs
4. Changing character of the HA
5. ***Recurrent a.m HAs*** or HAs that awaken or incapacitate the child
6. Short stature/deceleration of linear growth
7. Dx of Neurofibromatosis
8. Previous leukemia or CNS radiation

17

Lymph nodes:
-Large=?
-Common cause of enlargement?

***Lymph Nodes are considered large if > 10 mm***; exceptions:
1. Epitrochlear nodes > 5 mm
2. Inguinal node > 15 mm
***Most enlarged lymph nodes in children are related to infections:***
-Bacterial: Staph & Strep
-Atypical mycobacterium
-Cat scratch disease
-Viral: EBV & other herpes viruses

18

*Lymph node biopsy is Suggested by the following S/S

1. Enlarging nodes after 2-3 weeks of antibiotics
2. Nodes that aren't enlarging, but haven't diminished in 6-8 weeks
3. Nodes associated w/ any abnormal CXR
4. Adenopathy w/ associated weight loss, hepatosplenomegaly, unexplained fevers, and/or drenching night sweats
5. Adenopathy in the posterior auricular, epitrochlear or supraclavicular areas

19

Evaluating masses

Abdominal, Thoracic & Soft Tissue Masses (without a traumatic explanation) all require evaluation

20

Bone/Joint pain & cancer

-Most cancer pain is caused by bone, nerve or visceral involvement or encroachment
***-Bone pain is usually not an early symptom of cancer except for bone malignancies
(Ewing's sarcoma, osteosarcoma)
-Come & go early on disappearing for weeks-months
-Bone or joint pain is a presenting symptom in about 30% of ALL pts with
***-Can be confused w/ rheumatic diseases***

21

*Bone/Joint pain evaluation should be performed when:

1. Bone/joint pain is persistent
2. Associated with swelling/mass
3. Limited mobility or joint motion
4. Consistently wakes from sleep
5. Not relieved by NSAIDs

22

***Most common malignancy in childhood

ALL

23

Populations most at risk for ALL -

-2-5yr
- Males
- Caucasians
**-Hispanics*

24

**Childhood leukemia S/S

Bone marrow infiltration:
***1. Anemia
-Pallor, lethargy
-Dyspnea, murmur
***2. ↓ Platelets
-Bleeding, petechiae, purpura
***3. Neutropenia
-Fevers and infections
***4. Bone pain
-Limp, ↓walking, irritability
***5. Extrameduallary Disease
***6. Fever of Malignancy

25

Symptoms of extramedullary disease

-Lymphadenopathy, Hepatosplenomegaly
-Orthopnea, Cough
-Mediastinal mass, tracheal compression
-Facial nerve palsy
-Testicular enlargement
-Skin lesions, Gingival hypertrophy

26

*CBC findings in ALL

**-50% will have ↑WBC
**-80% will have lymphoblasts on the peripheral smear
**-95% will have >2 Cytopenias
-Only 4% will have 1 cytopenia
-Only 1% will have a normal CBC & differential

27

Common diseases mimicking leukemia S/S

1. Mononucleosis (EBV)
2. Acute anemia
3. Parvovirus B19
4. Idiopathic thrombocytopenia (ITP)

28

Risk factors for ALL

***1. Down syndrome
2. Prenatal exposure to X-rays
3. Postnatal exposure to high doses of radiation

29

*Precursor B-cell ALL
-Frequency?
-Presentation?

-80-85% of ALL's
-Presentation- ***Primarily BM involvement***
-Extramedullary involvement frequent
(CNS, skin, gonads, lymph nodes, liver, spleen)

30

*Precursor T-cell ALL
-Who is affected?
-Presentation?

~15% childhood ALL
-Adolescents > young children
-Males>Females
1. Mediastinal mass (50%-70%), ↑ WBC
2. Lymph nodes, CNS, skin, liver, spleen, gonads

31

NCI ROME leukemia risk criteria

Standard risk:
-Age: 1 - 10 years
-WBC: 10y
-WBC: >50,000

32

*ALL tx

*Chemotherapy: IV, oral, IT Intrathecally - into CSF), and IM
-Induction, Consolidation, Interim. Main (2), -Delayed intensification & Maintenance
-Average Risk: Girls & Boys-2½ years
-Higher Risk: Boys-3½ years
-Goals of our current clinical trials
-CNS prophylaxis in T cell or those CNS+
-F/U for 5yr after chemo, then annually for 10yr

33

*ALL prognosis -

->95% remission
- 75-85% survive w/o recurrence at least 5 years from Dx

34

*Late effects of ALL tx

1. Osteonecrosis, Osteopenia
2. Neuropathies
3. Neuropsychometric concerns
4. Obesity

35

AML Tx, outcomes & late e ffects of Tx:

Cytarabine, Dauno, VP-16 + BMT
Outcome: EFS remains at approximately 50%
Late Effects: Cardiac toxicity, 2nd malignancies, endocrinopathies, etc

36

*Most common solid tumor of childhood

Brain tumors
=20% of childhood malignancies

37

Most common brain tumors -

Astrocytoma
-Grade 1 - "JPA" - seen in association with NF, malignant and by location

38

*Symptoms of ↑Intracranial P. (brain tumors)

1. HA, Nausea/vomiting (am)
2. Double vision
3. Head tilt
4. ↓ Alertness, Lethargy/irritability
5. Poor feeding, FTT
6. Endocrine dysfunction
7. Unexplained behavior changes:
affect, motivation, energy level

39

*Signs of ↑Intracranial P. (brain tumors)

1. Papilledema, optic atrophy
2. Loss of vision
3. OFC (head circumference) increased
4. Bulging fontanelles, spreading sutures
5. "Setting sun" sign (Parinaud syndrome)
6. ↑ BP, ↓ pulse-> herniation?

40

Posterior Fossa primary tumor presentation

1. Ataxia & Tremors
2. Dysarthria & Stiff neck
3. Papilledema

41

Brainstem primary tumor presentation

1. Extremity weakness
2. Cranial nerve signs
-Double vision
-Facial weakness
-Swallowing dysfunction

42

Hemispheric Tumors presentation

1. Hemiparesis
2. Hemianopsia
3. Aphasia
4. Seizures

43

*Work up for brain tumors

1. CT: 65-95% sensitivity to detect brain tumors
***2. MRI: with and without contrast
3. CSF examination
4. Biopsy or preferred excision biopsy/resection

44

*Brain tumor tx

***1. Primarily Surgery (Gross total resection)
-Exception: Optic glioma
***2. Radiation: Potential in all tumors
-Exception: choroid plexus tumors
***3. Chemo: Adjunct in most
-Particularly in GCT (germ cell tumor), medulloblastoma

45

*Risk factors for brain tumors

***1. Astrocytomas= associated w/ NF-1 in 50-80%
2. Li-Fraumeni syndrome
-Germline mutation p53 tumor suppressor
3. Radiation to head & neck
-Survivors of childhood leukemias!

46

****Leading cause of morbidity & mortality in pediatric cancers

Brain tumors

47

***2nd most common abdominal malignancy in children.

Pathogenesis?

Wilms' Tumor

WT1 mutation: Tumor suppressor gene critical for normal renal development; Mutations predispose to embryonal tumor formation

48

*Presentation of Wilms' tumor

1. *Large palpable abdominal mass**
2. HTN, gross hematuria & fever in 5-30%

49

*Evaluation of Wilms' tumor

1. CBC, CMP -
2. CT Abdomen (Assess renal function prior to contrast - CREATININE!)
3. Chest XR & CT
4. Abdominal Ultrasound

50

*Tx of Wilms' tumor
-Prognosis?

Stage 1=nephrectomy
Stage 2+= Nephrectomy + Chemotherapy + Radiation

80-90% survival w/ multi-modal therapy

51

*Retinoblastoma:
-Epidemiology
-Pathogenesis
-Tx & prognosis

-80% occur in children <3
-30% are bilateral

-Loss of both alleles of the RB gene
-60% spontaneous, 40% hereditary=FAMILY HISTORY SUPER IMPORTANT!
-Tx: radiation, chemotherapy, enucleation
-85% long term survival

52

***Neuroblastoma:
-Epidemiology
-Findings
-Tx

***-Most common extra-cranial solid tumor in children
-75% are <4 yr (Average=18Months)***
more aggresive if 18+ months
-Findings: abdominal mass, pallor, weakness, bone pain, fever & weight loss
-FAMILY HISTORY SUPER IMPORTANT!
-TX: chemotherapy, surgery, radiation, stem cell transplant

53

*Poor prognostic indicators for neuroblastoma

1. Age >18 months
2. Myc-N amplification
3. Unfavorable Histology

54

*Neuroblastoma work up

1. Urine Catecholamines: HVA, VMA
2. Imaging Studies: CT, bone scan, MIBG scan
3. Bone Marrow Biopsies
4. Lymph Node evaluation

55

Botryoid (grape-like) vaginal mass

Rhabdomyosarcoma

S/S depend on age & site

56

*Ewing's Sarcoma & Osteosarcoma:
-Epidemiology
-Presentation
-Prognosis

***-Peak in adolescents ***
***-More common in males ***
***-Pain, swelling, often following sports injury ***
-Staging directly related to prognosis (very variable)

-treatment: limb salvage important!

57

Hodgkin's Disease:
-Age
-Clinical features -Age:

adolescents >> young child
-Painless lymphadenopathy
-Progresses over weeks- months
-95% Located in:
1. Cervical/supraclavicular ↑ LNS
(Unilateral or bilateral)
2. Mediastinum ± hilum
3. LN below diaphragm and spleen

58

*Hodgkins disease symptoms

Systemic "B" symptoms (first 3):
1. Fevers
2. Night sweats
3. Weight loss
4. Pruritus

59

most difficult area to get imaging done on a kid?

posterior fossa

60

astrocytomas are strongly associated with what?

NF-1

61

Wilms tumor more/less likely in which races?
Ages?

more likely in AA
less likely in Asians

rare in children>5

62

neuroblastoma location?

any neural crest tissue
-adrenal
-paraspinal sympathetic tissue: cervical, thoracic, pelvic

63

signs and symptoms of neuroblastoma:

clincal presentation?

-irritability
-weight loss
-bone pain
-fever
-RACCOON EYES -periorbital ecchymoses
-Proptosis
-bone leisions

-large abdominal mass - often crosses midline
-lower extremity weakness-spinal cord compression
-cervical high thoracic mass--> HORNERS SYNDROME (MIOSIS, PTOSIS,ANHYDROSIS)

64

Racoon eyes associated with what disease?

Neuroblastoma

65

most common extra cranial solid tumor in children?

neuroblastoma

66

myc-N associated neuroblastma.. prognosis?

...eh not good bud

67

neuroblastoma staging:

-1- localized tumor, complete excision
-2A- unilateral, incomplete gross resection; negative microscopic nodes
-2B- unilateral, positive ipsilateral nodes; negative contralateral
-3- across midline, or contralateral nodes
-4- dissemination: BM, liver, skin bones
-4S- <1yrs: local stage 1-2 with mets to BM liver and skin

68

most common soft tissue sarcoma in children:

rhabdomyosarcoma

69

rhabdomyosarcoma
-arrises from?
-origination?
-incidence peaks when?
-survival related to?

-arises from undifferentiated mesenchymal cells that differentiate into muscle

-originate anywhere

-peaks bw age 2-6

-survival directly related to staging higher stage=higher risk of death