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Pediatrics > Pediatric Cancer: An Overview > Flashcards

Pediatric Cancer: An Overview Flashcards

1
Q

Peak incidence of cancer is when for the first 14 years?

A

1st year of life

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2
Q

Common Childhood Cancers (0-14 years)
9
(most common)

A 
1, Leukemia 			32% 
2 CNS 				20
3, Lymphoma 			11
4. Neuroblastoma 		8
5. Soft Tissue Sarcomas	7
6. Kidney (Wilms)		6
7. Bone tumors			6
8. Germ-cell tumors 		4
9. Retinoblastoma 		3
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3
Q

Common cancers 15-19 years?
6
(most common)

A
  1. Lymphoma 25%
  2. Germ Cell 14%
  3. Leukemia 12%
  4. CNS 10%
  5. Soft Tissue Sarcoma 8%
  6. Bone 8%
  7. Thyroid Carcinoma 7%
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4
Q

Some syndromes are associated with increased risk of cancer in children. Such as?
3

A
  1. Down Syndrome
  2. Beckwith-Wiedeman
  3. Li Fraumeni Syndrome
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5
Q

Name the following cancers associated with each syndrome:

  1. Down Syndrome 3
  2. Beckwith-Wiedeman 5
  3. Li Fraumeni Syndrome 5
A

Down Syndrome

  1. ALL (12 x ↑risk)
  2. AML (46 x ↑risk)
  3. AMKL (466 x ↑risk)

Beckwith-Wiedeman

  1. Wilm’s Tumor in 4-20%
  2. Hepatoblastoma
  3. Rhabdomyosarcoma
  4. Adrenocortical carcinoma
  5. Neuroblastoma reported

Li Fraumeni Syndrome

  1. Bone tumor
  2. Soft tissue sarcomas
  3. Breast cancer
  4. Brain tumors
  5. Adrenocortical carcinoma
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6
Q

Tumors Associated with Genetic Syndromes

  1. Brain tumors? 5
  2. Kidney tumors? 7
  3. Bone Tumor? 5
  4. Liver Tumor? 8
A 
Brain Tumors
1. Neurofibromatosis
2. Tuberous Sclerosis
3. Turcot Syndrome
4, Von Hipple Lindau
5. Gorlin

Kidney Tumors

  1. Congenital Aniridia,
  2. Hemihypertrophy,
  3. GU malformations
  4. WAGR
  5. Denys-Drash
  6. Frasier
  7. Beckwith-Wiedemann
Bone Tumors
1. Hereditary retinoblastoma 
2 Paget’s disease 
3. Rothmund-Thompson
4. Li-Fraumeni syndrome
5. Poikiloderma, small stature, 
	  skeletal dysplasias

Liver Tumors

  1. Polyposis
  2. Gardner
  3. Beckwith-Wiedeman
  4. Hemi-hypertrophy
  5. Tyrosinemia
  6. Glucogen storage dx
  7. α-1 anti-trypsin
  8. Hemochromotosis
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7
Q

Uncommon Symptom: Match with the possible diagnosis

  1. Recurrent fever & bone pain?
  2. Morning headache with vomiting?
  3. Neck mass not responding to abx?
  4. Chronic bone pain, swelling, limp?
  5. Progressive abdominal distension?
  6. Bleeding, bruising, pallor, fatigue?
  7. Combined cytopenias?
A
  1. Leukemia
  2. CNS tumor
  3. Lymphoma
  4. Bone tumor
  5. Abdominal pain
  6. Laukemia
  7. Leukemia, marrow failure
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8
Q

Presenting symptoms of Leukemia

  1. What are the symptoms due to?
  2. What are these symptoms? 4
A
  1. Symptoms due to bone marrow replacement by dysfunctional immune cells
    • Bone pain,
    • anemia and fatigue,
    • fevers and infection,
    • petechiae and bleeding
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9
Q

Brain Tumors: Symptoms from increased pressure and location

  1. In a child? 4
  2. Infant? 4
  3. Specific sites? 4
A

Child

  1. Headaches (morning)
  2. Vomiting
  3. Decreased academics
  4. Personality changes

Infant

  1. Large head, bulging fontanelle
  2. Anorexia, FTT
  3. Loss of milestones
  4. Irritable, shrill cry

Specific Sites

  1. Vision change
  2. Seizure (
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10
Q

Neuro-onc emergency

  1. normal ICP?
  2. decreased blood flow and symptoms?
  3. Often leads to unconsciousness?
  4. Over this level causes what? 3
A
  1. 10-18 mmHg
  2. 25-40
  3. > 40
    • Stroke,
    • herniation,
    • death
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11
Q

Lymphomas can present with either local and/or systemic signs.

  1. Localized? 3
  2. Systemic? 3
A
  1. Localized
    - Painless enlarged lymph nodes
    - Abdominal pain or mass
    - Emergent: compromise of vital structure
  2. Systemic
    - Fevers, night sweats, weight loss, fatigue
    - Pruritus,
    - Pain with ETOH ingestion
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12
Q

Superior vena cava or superior mediastinal syndromes are considered how serious?

Present how?

A

Medical Emergencies

Present with really big head

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13
Q

Why do we have to be careful with sedation with mediastinal mass?

A
  1. Sedation can cause fatal resp failure
    - Anesthesia can aggravate SVC/airway compression
    - Difficult to intubate past obstruction
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14
Q
  1. Obtain tissue by the least invasive means possible
    Emergency management may include what?
  2. Biopsy may be uninterpretable in as little as _____?
A
  1. steroids or radiation

2. 48hrs

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15
Q

Abdominal Tumors

  1. On physical exam? 2
  2. Symptoms? 11
A
    • Distension,
    • palpable mass (clothes don’t fit)
    • Nausea,
    • vomiting,
    • diarrhea,
    • constipation,
    • weight loss,
    • fever
    • Obstruction,
    • pain,
    • jaundice,
    • blood in the urine,
    • renal failure
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16
Q

Bone tumor symptoms?

7

A
  1. Pain / tenderness / limp
  2. Soft tissue mass
  3. Night pain
  4. “Growing pains”
  5. History of minor trauma
  6. Pathologic fracture
  7. Pain often present
    for 2-3 months
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17
Q

What are the types of ALL?

3

A
  1. Precursor B cell (B-ALL)
  2. Burkitts (mature B-ALL, treated like lymphoma)
  3. T cell (T ALL)
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18
Q
  1. HOw do we diagnose AML?

2. Risk factors? 4

A

WHO is clinical/molecular.
1. Marrow with 20% blasts or more,

    • prior therapy,
    • Down syndrome,
    • MDS,
    • cytogenetics
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19
Q

Cancer predisposition syndromes

4

A
  1. Li Fraumeni (SBLA): Tp53 and other genes
  2. Ataxia Telangectasia (T ALL, T NHL)
  3. Neurofibromatosis Type 1 (AML»ALL)
  4. Bloom Syndrome (leuk, lymphoma, AML>ALL)
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20
Q

Predisposing conditions: Congenital anomaly syndromes 1

A

Klinefelter’s

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21
Q

Congenital and aquired immunodeficiencies

4

A
  1. Ataxia telangectasia,
  2. Wiskott aldrich synd,
  3. chronic immunosuppression, 4. AIDS
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22
Q

Down syndrome

  1. Percent increase in leukemia?
  2. Most common type of leukemia?
  3. Risk period limited to what?
A
  1. 10-20x increased risk of leukemia
  2. Relative risk of AML higher but ALL more common except during first year of life
  3. Risk period limited to first three decades
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23
Q

Acute Lymphoblastic Leukemia

1. Most common type?

A
  1. 80-85% cases are B-precursor
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24
Q

Acute Lymphoblastic Leukemia: Incidence peaks when?

A

Incidence peaks at 2-4 yrs in developing countries

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25
Q

Leukemia Presentation

Symptoms?

A
  1. Bone pain (35%)
  2. Adenopathy
  3. Hepatosplenomegaly
  4. Thymic expansion
    Mediastinal mass esp T ALL
  5. CNS sx
    Headache
    Neck pain
    CN palsy
    Seizures
  6. Testicular involvement
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26
Q

Leukemia Presentation:

  1. Bone pain is due to what?
  2. Tymic expanision is often caused by what?
  3. CNS symptoms? 4
  4. Describe the testicular involvement in leukemia? 2
A
  1. Due to expansion of marrow cavity
  2. Mediastinal mass esp T ALL
    • Headache
    • Neck pain
    • CN palsy
    • Seizures
    • Painless, enlarged, usually rock hard testes
    • Must confirm with biopsy
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27
Q

Hematologic Findings and Associated Clinical Findings

7

A
  1. WBC can be high, normal or low
  2. Decreased neutrophil # and
  3. FUNCTION: infection
  4. Risk of Tumor lysis syndrome with high WBC (>50)
  5. Hyperleukocytosis (WBC >250-500k) may be associated with CNS and pulmonary sx but much lower risk with ALL compared to AML
  6. Decreased platelets: bruising, bleeding and petechiae
  7. Decreased RBC: pallor and fatigue
28
Q

Pancytopenia
DDx?
9

A
  1. Leukemia
  2. Aplastic Anemia (acquired, congenital, viral assoc)
  3. Metastatic Solid tumor
  4. Myelodysplastic syndrome
  5. Hemophagocytic Lymphohistiocytosis
  6. Infection, post infectious (EBV especially)
  7. Systemic Lupus Erythematosus
  8. Hypersplenism
  9. B12 or Folate deficiency
29
Q

Hepatosplenomegaly and Pancytopenia
DDx?
7

A
  1. Leukemia (AML and ALL)
  2. Hepatitis Associated
  3. Aplastic Anemia
  4. Metastatic Solid Tumor
  5. MDS
  6. HLH
  7. SLE
30
Q
  1. ALL can mimic what?

2. Strongest factors predictive of ALL are what? 3

A
  1. Juvenile Rheumatoid Arthritis
    • low WBC (
31
Q 
Immune Thrombocytopenia (ITP) differentiates from Leukemia how?
2
A
  1. Most ITP kids are very healthy and have Acute onset of bruising
  2. ITP typically nml Hgb and WBC and plts
32
Q

Acute Lymphocytosis
DDx?
6

A
  1. Infection (esp pertussis, EBV, CMV)
  2. ALL
  3. Thyrotoxicosis
  4. Neutropenias (relative lymphocytosis)
  5. Addison’s Disease (relative lymphocytosis)
33
Q

Lymphadenopathy
DDx?
5

A
  1. Infection
  2. Metastatic disease
  3. Leukemia
  4. Lymphoma
  5. Autoimmune Disease (JRA, SLE)
34
Q

Diagnosis of Leukemia?
7

Sanctuary Sites (CNS and testes)?
2
A
  1. CBC with differential,
  2. Peripheral Smear
  3. Bone Marrow Aspirate,
  4. lumbar puncture
  5. Flow cytometry,
  6. morphology
  7. cytogenetics
  8. LP,
  9. testicular exam
35
Q

Treatment: Standard risk ALL?

6

A
  1. 4 week 3 drug induction
  2. 4 week oral consolidation with IT
  3. 8 week Int Maint
  4. 8 week Delayed Intensification
  5. 2nd Int Maint
  6. Maintenance (2 +yrs)
36
Q

HR ALL and T ALL

6

A
  1. 4 week 4 drug induction
  2. 8 week IV consolidation
  3. 8 week Int Maint
  4. 8 week Delayed Intens
  5. 2nd Interim Maint
  6. Maintenance (2+yrs)
37
Q

Leukemia/Lymphoma

  1. What do we do with poor response to therapy?
  2. CNS 3 disease typically receives what?
  3. Many ______ also receive regardless of CNS status
  4. ________chemotherapy has been key to improving outcomes
A
  1. alters treatment regimen
  2. Cranial Irradiation.
  3. T ALL
  4. Intrathecal
38
Q

Leukemia/Lymphoma
Relapse: What factors go into relaspse?
4

A
  1. Site of relapse important
  2. Time to relapse
  3. Age at initial dx
  4. Genetic Features
39
Q

Leukemia/Lymphoma
1. Site of relapse important: What is the worst?

  1. Time to relapse: Early or late is worse?
  2. Age at initial dx: Poor outcome for who?
  3. Genetic Features: Which cancer does well and which does not?
A
  1. Bone marrow worse than extramedullary (resistant disease)
  2. Earlier much worse than later, worse less than 18 months since dx
  3. Very poor outcomes for teenagers who then relapse
    • Marrow relapse of T ALL has very poor outcome
    • Subset of Low/Standard risk ALL does relatively well
40
Q

Acute Myeloid Leukemia

  1. Peaks at what age?
  2. What is an important sign?
  3. Prognosis?
A
  1. Peaks in infancy and teen yrs
  2. Cytogenetic lesions important
  3. Worse prognosis than ALL, 5y EFS 60-70%
41
Q

Presentation of AML?

12

A
  1. Fever 33%
  2. Bone Pain 20%
  3. LAD 10-20%
  4. HSM 50%
  5. Extramedullary dz
  6. Chloromas
  7. Sepsis
  8. Hyperleukocytosis
  9. Anemia
  10. Thrombocytopenia 75%
42
Q

Pathogonomic to AML?

A

Auer Rod in AML blast cell

43
Q

Risk Groups for AML?

4

A
  1. Down syndrome less than 4 treated with less intense chemotherapy
  2. APML treated with All trans retinoic acid (ATRA) and Arsenic Trioxide
  3. Favorable four courses of intense chemotherapy
  4. Unfavorable to Bone marrow transplant if acceptable donor found after two rounds of chemotherapy
44
Q

Treatment: AML
1. Describe remission Induction therapy?

  1. Post Remission consolidation. What are our options?
  2. CNS prophylaxis: Compared to ALL?
  3. Maintenance therapy?
  4. No_______ even with CNS disease
A
  1. Two course intense chemo, Very high risk of invasive infection
  2. Transplant for High risk
    Or two courses of intense chemotherapy
  3. Much less intense than ALL due to low rate of CNS relapse. If CNS positive weekly IT chemo until clears
  4. Survival benefit NEVER demonstrated so no maint course
  5. Irradiation
45
Q

Hodgkin Lymphoma
1. Classical HL characteristics? 4

  1. What kind is predominant?
A
    • Nodular sclerosing
    • Mixed cellularity
    • Lymphocyte predominant
    • Lymphocyte depleted
  2. Nodular Lymphocyte
46
Q

Non-Hodgkin Lymphoma

types?

A
  1. Burkitt
  2. Lymphoblastic
  3. Diffuse large B cell
  4. Anaplastic large cell
47
Q

Hodgkin lymphoma

  1. Presentation? 3
  2. B symptoms (Prognostic in HL only)? 3
  3. Other sx (not prognostic)? 6
  4. Lab findings?
A
    • Painless lymphadenopathy,
    • mediastinal mass, and/or
    • constitutional symptoms
    • weight loss (>10% in 6 months), -Drenching night sweats,
    • unexplained fevers (>38*C) for 3 consecutive days
    • fatigue,
    • anorexia,
    • mild weight loss,
    • pain following alcohol
    • ingestion,
    • pruritis
    • elevated inflammatory markers: CRP, ESR, ferritin, Copper; anemia of chronic inflammation and possible immune dysregulation (autoimmune neutropenia, autoimmune hemolytic anemia, immune thrombocytopenia or nephrotic syndrome)
48
Q

HL: DDx Non-malignant

7

A
  1. Normal thymus
  2. EBV
  3. Atypical mycobacterium
  4. Histoplasmosis
  5. toxoplasmosis
  6. Lymphoproliferative disorder
  7. Progressive transformation of germinal centers
49
Q

HL: DDx Malignant?

4

A
  1. NHL
  2. Germ Cell tumor
  3. Soft tissue Sarcoma
  4. Metastasis
50
Q

HL: Diagnosis
Labs? 3
Imaging? 4

If higher stage will need to do what?

A
  1. CBC,
  2. CMP,
  3. inflammatory markers
  4. CXR
  5. CT neck and chest
  6. CT or MRI of abdomen and pelvis
  7. FDG-PET
  8. bilateral bone marrow biopsy and aspirate to eval for metastatic dz
51
Q

HL:

  1. Risk stratification based on what?
  2. Bulk disease is how much of the thoracic diameter on PA chest X-ray or how much for a nonmediastinal mass?
  3. What is key in the diagnosis?
  4. Malignant cells are which? 2
  5. Phenotype can determine histology so what is important for this?
  6. What is implicated in some but not all?
A
    • staging,
    • presence or
    • absence of B symptoms and bulk disease.
  2. is >1/3 thoracic diameter on PA CXR or >6cm nonmediastinal mass
  3. Biopsy of tissue key. Excisional LN biopsy

4.

  • Hodgkin Reed Sternberg cells (several variations),
  • Lymphocytic & Histiocytic Cells
  1. Immunostaining of biopsy very important.
  2. EBV implicated in some HL but not all
52
Q

Treatment for HL

  1. Multinodal?
    - What is the prognosis?
A
  1. Multi-agent chemotherapy and radiation standard

- Survival >90% even for advanced disease

53
Q

Risk factors for NHL?

4

A
  1. EBV (for some),
  2. immunodeficiency,
  3. immunosuppression,
  4. pesticides
54
Q

Presentation similar to HL
Emergency Presentations are frequent
8

A
  1. Compression of airway due to superior mediastinal syndrome
  2. Pulmonary effusions cause breathing complications
  3. CV: SVC syndrome, tamponade, arrhythmia
  4. Paraspinal/epidural mass complications
  5. Electrolyte abnormalities due to tumor lysis
  6. Organ failure: renal compromise due to compression 2/2 mass
  7. GI obstruction, intussusception, bleeding, perforation, jaundice and pancreatitis
  8. Cytopenias due to marrow infiltration
55
Q

NHL presentation:

  1. Endemic Burkitt? 4
  2. Sporadic Burkitt? 5
  3. DLBCL? 2
  4. Lymphoblastic? 5
  5. ALCL? 3
A
  1. jaw swelling,
  2. abdominal or orbital swelling,
  3. paraspinal mass,
  4. CNS or marrow involvement
  5. rapidly expanding abd mass, 2. spontaneous tumor lysis,
  6. non specific gi sx,
  7. CNS involvment,
  8. marrow infiltation
  9. more diverse (LN, liver, spleen, marrow mediastinum), 2. extranodal involvement (bowel, mesentery, retroperitoneum)
  10. mediastinal mass,
  11. pleural/pericardial effusion, 3. pain,
  12. dysphagia,
  13. dyspnea
  14. slowly progressive,
  15. systemic sx,
  16. organs and skin can be involved.
56
Q

Treatment

  1. All recieve what?
  2. Most recieve what?
  3. Most do not recieve what?
  4. Burkitt leukemia is treated like what?
A
  1. All receive chemotherapy
  2. Most receive CNS ppx
    Lymphoblastic lymphoma treated like ALL, long therapy with maintenance
  3. Most do not receive irradiation (exception is CNS + lyphomblastic lymphoma)
  4. Burkitt leukemia treated like Burkitt lymphoma
57
Q 
Prognosis
Burkitt?
DLBCL?
ALCL?
Burkitt leukemia?
A 
Overall survival is high
Burkitt 90%
DLBCL 90%
ALCL 85%
Burkitt leukemia 80%
58
Q
  1. Whats the Second most common solid neoplasm in childhood?
  2. Median age?
  3. Originates where?
  4. Can metastasize?
A
  1. Neuroblastoma
  2. Median age 22 months
  3. Originates from neural crest tissue
  4. Can metastasize to bone, bone marrow, lymph nodes
59
Q

Genetics
1. Familial neuroblastoma associated with what?
2. Other things its associated with?
5

A
  1. Familial neuroblastoma associated with an anaplastic lymphoma kinase (ALK) germline mutation
    • Noonan syndrome
    • Congenital cardiac defects
    • Constitutional deletion of chr 1p
    • Neurofibromatosis
    • Neurocristopathy syndrome
60
Q

NBL presentation

A

Periorbital echymosis

61
Q

Presentation NBL

10

A
  1. Coindicental finding (30%)
  2. Neck mass
  3. GU sx
  4. Neurologic abnormalities
  5. Paraneoplastic syndromes
    - Opsoclonus/myoclonus (4%)
    - Secretory diarrhea due to vasoactive intestinal peptide
  6. Ill appearing: malaise, irritable, weight loss, anorexia
  7. Pain: ill defined (40%)
  8. Abdominal distension (35%)
  9. Proptosis, raccoon eyes
  10. Fever, resp distress
62
Q

NBL: Diagnosis

6

A
  1. CT/MRI scan (spine)
  2. Eval primary and nodal metastatic sites
  3. Bilateral Bone marrow biospy and aspirate
  4. Radiolabelled MIBG (I-123)
    If non-avid (10%) then do PET
  5. Catecholamine metabolites: vanillylmandelic acid (VMA) and homovanillic acid (HVA) 90%
  6. Tumor genetic analysis
    - Tumor biopsy
    - Histologic grading
63
Q

NBL: Prognostic Criteria

7

A
  1. Independent Variables
  2. All poor prognostic characteristics
  3. Age >18months
  4. Advanced stage dz
  5. Myc-N amplification
  6. Poorly or undifferentiated tumor histology
  7. Diploid DNA content
64
Q
  1. Wilms Tumor: What kind of tumor?
A
  1. Nephroblastoma
65
Q

Associated Malformations and Genetic Predisposition

5

A
  1. Aniridia 1%
  2. Hemi-hypertrophy 2%
  3. GU malformation 6%
  4. Malformation of any type 13%
  5. Children with associated malformations or bilateral disease are dx earlier
66
Q

Presentation?

A
  1. Usally presents as an asymptomatic upper abdominal mass
    Abdominal pain (20-30%)
    Fever (20-30%)
    Anemia (bleed into tumor)
    Hematuria (tumor extension into renal pelvis) (20-30%)
    Hypertension (distortion of renal vasculature) (25%)

Pediatrics (18 decks)

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