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Heme Exam 3 > Ped Oncology > Flashcards

Ped Oncology Flashcards

1
Q

number one cause of disease related death in children?

A

cancer bro

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2
Q

*Most common cancers of childhood?

A

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

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3
Q

is incidence of childhood cancer increasing?

A

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

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4
Q

5 yr survival for common bone marrow related cancers

A

lowest to highest:

  • AML
  • NB
  • ALL
  • Wilms
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5
Q

Epidemiology of childhood cancer

A

-1/330 Americans develops cancer before 20

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

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6
Q

Long-term effects of childhood cancer

A
  • 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
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7
Q

2 childhood cancers that may be increasing in incidence

A
  1. ALL

2. Brain & CNS

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8
Q

Most improved outcomes has been for which childhood cancer?

A

ALL

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9
Q

Where are children usually treated for cancer?

A

> 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

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10
Q

General points about causes of childhood cancers

A

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
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11
Q

Familial/genetic diseases associated with increased cancer risk:

A
  1. Neurofibromatosis
  2. Familial polyposis
  3. Li-Fraumeni syndrome
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12
Q

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

A
  1. Immune deficiencies
  2. Metabolic disorders
  3. Disorders of chromosome stability
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13
Q

*General points about presentation of childhood cancers

A
  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
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14
Q

*Red flags for Leukemia

A
  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)
  6. sudden weight loss
  7. hypertension
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15
Q

*Red flags for Brain Tumors

A

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

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16
Q

*Conditions Suggesting Radiographic Evaluation in Headaches

A
  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
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17
Q

Lymph nodes:

  • Large=?
  • Common cause of enlargement?
A
  • 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
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18
Q

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

A
  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
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19
Q

Evaluating masses

A

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

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20
Q

Bone/Joint pain & cancer

A

-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*

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21
Q

*Bone/Joint pain evaluation should be performed when:

A
  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
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22
Q

***Most common malignancy in childhood

A

ALL

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23
Q

Populations most at risk for ALL -

A
  • 2-5yr
  • Males
  • Caucasians
  • -Hispanics
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24
Q

**Childhood leukemia S/S

A

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
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25
Q

Symptoms of extramedullary disease

A
  • Lymphadenopathy, Hepatosplenomegaly
  • Orthopnea, Cough
  • Mediastinal mass, tracheal compression
  • Facial nerve palsy
  • Testicular enlargement
  • Skin lesions, Gingival hypertrophy
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26
Q

*CBC findings in ALL

A
  • *-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
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27
Q

Common diseases mimicking leukemia S/S

A
  1. Mononucleosis (EBV)
  2. Acute anemia
  3. Parvovirus B19
  4. Idiopathic thrombocytopenia (ITP)
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28
Q

Risk factors for ALL

A
  • **1. Down syndrome
    2. Prenatal exposure to X-rays
    3. Postnatal exposure to high doses of radiation
29
Q
  • Precursor B-cell ALL
  • Frequency?
  • Presentation?
A

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

30
Q
  • Precursor T-cell ALL
  • Who is affected?
  • Presentation?
A

~15% childhood ALL

  • Adolescents > young children
  • Males>Females
    1. Mediastinal mass (50%-70%), ↑ WBC
    2. Lymph nodes, CNS, skin, liver, spleen, gonads
31
Q

NCI ROME leukemia risk criteria

A

Standard risk:

  • Age: 1 - 10 years
  • WBC: 10y
  • WBC: >50,000
32
Q

*ALL tx

A
  • 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
Q

*ALL prognosis -

A
  • > 95% remission

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

34
Q

*Late effects of ALL tx

A
  1. Osteonecrosis, Osteopenia
  2. Neuropathies
  3. Neuropsychometric concerns
  4. Obesity
35
Q

AML Tx, outcomes & late e ffects of Tx:

A

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

36
Q

*Most common solid tumor of childhood

A

Brain tumors

=20% of childhood malignancies

37
Q

Most common brain tumors -

A

Astrocytoma

-Grade 1 - “JPA” - seen in association with NF, malignant and by location

38
Q

*Symptoms of ↑Intracranial P. (brain tumors)

A
  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
Q

*Signs of ↑Intracranial P. (brain tumors)

A
  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
Q

Posterior Fossa primary tumor presentation

A
  1. Ataxia & Tremors
  2. Dysarthria & Stiff neck
  3. Papilledema
41
Q

Brainstem primary tumor presentation

A
  1. Extremity weakness
  2. Cranial nerve signs
    - Double vision
    - Facial weakness
    - Swallowing dysfunction
42
Q

Hemispheric Tumors presentation

A
  1. Hemiparesis
  2. Hemianopsia
  3. Aphasia
  4. Seizures
43
Q

*Work up for brain tumors

A
  1. CT: 65-95% sensitivity to detect brain tumors
    * **2. MRI: with and without contrast
  2. CSF examination
  3. Biopsy or preferred excision biopsy/resection
44
Q

*Brain tumor tx

A
  • **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
Q

*Risk factors for brain tumors

A
  • **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
Q

**Leading cause of morbidity & mortality in pediatric cancers

A

Brain tumors

47
Q

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

Pathogenesis?

A

Wilms’ Tumor

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

48
Q

*Presentation of Wilms’ tumor

A
  1. *Large palpable abdominal mass**

2. HTN, gross hematuria & fever in 5-30%

49
Q

*Evaluation of Wilms’ tumor

A
  1. CBC, CMP -
  2. CT Abdomen (Assess renal function prior to contrast - CREATININE!)
  3. Chest XR & CT
  4. Abdominal Ultrasound
50
Q
  • Tx of Wilms’ tumor

- Prognosis?

A

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

80-90% survival w/ multi-modal therapy

51
Q
  • Retinoblastoma:
  • Epidemiology
  • Pathogenesis
  • Tx & prognosis
A
  • 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
Q
  • **Neuroblastoma:
  • Epidemiology
  • Findings
  • Tx
A

-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
Q

*Poor prognostic indicators for neuroblastoma

A
  1. Age >18 months
  2. Myc-N amplification
  3. Unfavorable Histology
54
Q

*Neuroblastoma work up

A
  1. Urine Catecholamines: HVA, VMA
  2. Imaging Studies: CT, bone scan, MIBG scan
  3. Bone Marrow Biopsies
  4. Lymph Node evaluation
55
Q

Botryoid (grape-like) vaginal mass

A

Rhabdomyosarcoma

S/S depend on age & site

56
Q
  • Ewing’s Sarcoma & Osteosarcoma:
  • Epidemiology
  • Presentation
  • Prognosis
A
  • **-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
Q

Hodgkin’s Disease:

  • Age
  • Clinical features -Age:
A 
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
Q

*Hodgkins disease symptoms

A

Systemic “B” symptoms (first 3):

  1. Fevers
  2. Night sweats
  3. Weight loss
  4. Pruritus
59
Q

most difficult area to get imaging done on a kid?

A

posterior fossa

60
Q

astrocytomas are strongly associated with what?

A

NF-1

61
Q

Wilms tumor more/less likely in which races?

Ages?

A

more likely in AA
less likely in Asians

rare in children>5

62
Q

neuroblastoma location?

A

any neural crest tissue

  • adrenal
  • paraspinal sympathetic tissue: cervical, thoracic, pelvic
63
Q

signs and symptoms of neuroblastoma:

clincal presentation?

A
  • 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
Q

Racoon eyes associated with what disease?

A

Neuroblastoma

65
Q

most common extra cranial solid tumor in children?

A

neuroblastoma

66
Q

myc-N associated neuroblastma.. prognosis?

A

…eh not good bud

67
Q

neuroblastoma staging:

A
  • 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
Q

most common soft tissue sarcoma in children:

A

rhabdomyosarcoma

69
Q

rhabdomyosarcoma

  • arrises from?
  • origination?
  • incidence peaks when?
  • survival related to?
A
  • 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

Heme Exam 3 (8 decks)

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