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Pediatrics Flashcards

1
Q

ALL background and incidence -5

A

Most common pediatric cancer by far

2400 new cases/year in US

80% ALL and 20% AML

Peak incidence for ALL is 2-3 years of age

Cause is unknown

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2
Q

ALL signs and symptoms -8

common misdiagnosis is rheumatoid arthritis

A

WBC is normal or low in 59% of kids

Normochromic, normocytic anemia

Thrombocytopenia

Fever

Bleeding

Bone pain (limping, want to be carried)

Lymphadenopathy

Hepatosplenomegaly

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3
Q

ALL dx -3

A

Bone marrow aspirate and biopsy

If at least 25% of lymphoid cells in BM are blasts, the patient has ALL

MRD = MINIMAL RESIDUAL DISEASE is tested during induction

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4
Q

ALL tx - TLS -5

A

First, deal with tumor lysis issues

Hydrate (maintenance fluids = 1500ml/m2/day), give 1.5-2 times this rate

Alkalinization has fallen out of favor. Thought it kept uric acid in solution, but not shown to be helpful. however some places use it.

Allopurinol

Rasburicase if uric acid is >10mg/dl or Creatinine is elevated or WBC is greater than 100,000/mm3

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5
Q

ALL tx - TLS - At a glance method for evaluating creatinine: -3

A

Creatinine is normally 0.2mg/dl up to 2 years of age

Then you add 0.1mg/dl per year until they reach 8 years of age,

Then their creatinine values are the same as adults

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6
Q

ALL - Induction - key concepts -3

DOES RISK STRATIFICATION MATTER?

A

Lasts 4 weeks

Standard risk: 3 drugs (besides IT chemo)

High risk: 4 drugs (besides IT chemo)

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7
Q

ALL - Induction - high risk definition -4

A

kids less than 1 year of age or greater than 9 years of age,

presenting WBC > 50,000,

T-cell ALL,

certain translocations

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8
Q

ALL - Induction - 3 drug regimen -6

DEX INC RISK OF AVASCULAR NECROSIS IF PT >9YR

DOSING DURING INDUCTION IS NOT BASED ON BLOOD COUNTS

A

Prednisone 40mg/m2/day or dexamethasone 6mg/m2/day for 28 days

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9
Q

ALL - IT methotrexate -2

A

Dose is based on age with a max of 15mg for kids greater than or equal to 9 years of age

Example is MTX
1-1.99 years get 8mg

2-2.99 years get 10mg

3-8.99 years get 12mg

greater than or equal to 9 years get 15mg

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10
Q

ALL - Induction - 4 drug regimen -2

A

Use the 3 drug regimen, plus daunorubicin 25mg/m2 IV on days 2, 8 and +/- 15

If daunorubicin is used, then the steroid must be prednisone because the combo of daunorubicin and dexamethasone poses a greater risk of fungal infections

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11
Q

ALL - monitoring response -3

A

Do BM aspiration at day 7 or 14 and at day 28

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12
Q

ALL - Post-Remission Therapy: aka Consolidation, early or delayed intensification - concepts -5

IS OPTIMAL REGIMEN KNOWN?

WHAT IS INTENSITY AND DURATION OF TX BASED ON?

A

Begins if the patient is in remission after induction

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13
Q

ALL - Post-Remission Therapy: aka Consolidation, early or delayed intensification - regimens -6

PROBABLY NOT ON TEST - NO SOC

A

Methotrexate 1gm/m2 IV q3 weeks x 6 doses

Escalating dose methotrexate plus Pegaspargase

Cyclophosphamide1gm/m2 followed by cytarabine 75mg/m2 SQ or IV x 10 days with thioguanine x 14 days

Vincristine/pred pulses, doxorubicin, various doses of asparaginase

High risk kids get more aggressive treatment for a longer duration, regimens include high dose MTX 5gm/m2 and high dose asparaginase (25,000 units/m2) IM

Longer duration for boys due to risk of testicular relapse

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14
Q

ALL - Post-Remission Therapy: mainenance concepts -3

A

Without maintenance therapy, most kids with ALL will relapse - compliance also effects relapse

Can still have undetectable disease after induction and consolidation (MRD)

Trying to knock off cells coming out of G0 phase over a couple of years

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15
Q

ALL - Post-Remission Therapy: mainenance regimen -5

WHAT IS USED FOR DOSE ADJUSTMENT?

A

ANC’s are best to see if pt taking drug. used to dose adjust.

Methotrexate 20mg/m2 usually by mouth once a week x 1.5-2 years

Mercaptopurine 50-75mg/m2 daily x 1.5-2 years
Doses adjusted to keep ANC between 500-1500/mm3

Periodic IT methotrexate (example = every 16 weeks)

Periodic Vincristine x1 plus 7 day pred or dex pulses (VP pulse)

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16
Q

Relapsed ALL -2

WHERE DOES DZ RELAPSE?

A

CNS relapse – treat with cranial radiation and re-induction/re-consolidation therapy and then maintenance

BM relapse- if they relapse on therapy or within 1 year post chemo completion, BMT is a better option than more chemo. Chance of 2nd remission is good, but long term survival is often less than 50%

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17
Q

ALL -Radiation concepts -2

WHAT IS MAIN USE?

ADR?

A

Cranial XRT used in kids with T-cell ALL

Main use is for CNS relapse

Causes growth deficiency and impaired intellectual development

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18
Q

ALL prognosis -7

WHAT AGE IS BEST?

WHAT WBC IS BEST?

A

85-90% long term survival

Age 1-9 years is best

Initial WBC < 50K is favorable

MRD (if detected, is bad) is used to tailor post-remission therapy

favorable: hyperdiploid; trisomy 4, 10, or 17 have lower risk of relapse, TEL/AML1 t(12;21)

Unfavorable: t(9;22) = Philadelphia chromosome, MLL rearrangements: t(4;11), t(11;19) and t(1;11)

Early response; bone marrow remission on day 7 or 14 is favorable

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19
Q

AML regimen -3

A

Cytarabine 3000mg/m2 IV over 3 hours every 12 hours for 6 doses alternating with the next course at 100mg/m2/day continuous infusion for 7 days

Daunorubicin 45-60mg/m2 IV daily for 2-3 days per course

Courses repeated for 4-6 months

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20
Q

AML concepts -6

WHERE IS MOST COMMON RELAPSE?

DO YOU USE MAINTENANCE?

A

chemo is of shorter duration overall vs ALL, but it is much more toxic

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21
Q

AML: post induction tx -3

WHAT IS PREFERRED?

WHAT IS CONSOLIDATION VS INTENSIFICATION?

A

If the patient has a living related donor who matches, they go to BMT once in complete remission

Consolidation: if different agents (vs induction agents) are used

Intensification: if the same agents are used but at higher doses

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22
Q

AML prognosis (6pts)

A

50% long term survival

60% OS with HSCT from living related donor

Some subtypes have more favorable prognosis

Poor prognosis with monosomy 7, -5/5q-, or FLT3/ITD allelic ratio

BM blasts at day 15 and MRD of 1% or more after 1st induction are bad prognostic indicators

Age less than 1 and WBC > 20,000/mm3 have worse prognosis (3-10yo have best prognosis)

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23
Q

Late effects with ALL and AML (4pts)

A

anthracycline cardiotoxicity

secondary AML (for ALL patients who got epipodopylotoxins - LESS BECAUSE NOT BEING USED MUCH ANYMORE)

decreased bone mineral density (steroids)

leukoencephalopathy (high dose methotrexate) - lifetime learning problems

XRT neurocog problems LESS as this tx is avoided as much as possible, especially if <5yo

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24
Q

Pediatric CNS tumors (3 main types)

A

Medulloblastoma

Astrocytoma

Brain stem gliomas

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25
Q

Pediatric CNS tumors- Medulloblastoma -5

A

A primitive neuroectodermal tumor (PNET)

Median age at diagnosis = 5-6 years

Arises in cerebellum

25-45% have leptomeningeal mets at diagnosis

Rapidly growing, so short duration of symptoms - BECAUSE OF THIS AMENABLE TO CHEMO

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26
Q

Pediatric CNS tumors- Medulloblastoma - signs and sx -6

A

Early symptoms:
signs and symptoms of increased ICP, headache, emesis, lethargy, hydrocephalus

Late symptoms : 
ataxia, 
diplopia, 
weakness, 
papilledema, 
strabismus
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27
Q

Pediatric CNS tumors- Medulloblastoma - staging : favorable vs high risk -4

WHAT DOES RESIDUAL DZ MEAN TO RISK?

DOES AGE MATTER?

A

Favorable disease:
1. children older than 3 years of age,

  1. localized disease that has complete or near total resection

High risk:
1. invasive disease that limits total resection (greater than 1.5cm2 residual disease)

  1. overt metastatic disease (subarachnoid seeding)
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28
Q

Pediatric CNS tumors- Medulloblastoma - staging -Chang Classification (prognostic value) -5

M IS PROGNOSTIC NOT T

A

M0 = No gross subarachnoid or heme mets

M1 Microscopic tumor cells in CSF

M2 Gross nodular seeding in cerebellum, cerebral subarachnoid space, or in third or fourth ventricles

M3 Gross nodular seeding in spinal subarachnoid space

M4 Extraneuraxial metastasis

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29
Q

Pediatric CNS tumors- Medulloblastoma - treatment - surgery -3

TX IS SURGERY+XRT+CHEMO

A

Surgery is KEY! Need complete or near complete resection.

Can do post-op MRI to detect residual disease and operate a second time to improve resection.

Bone marrow aspirate and biopsy (5% have systemic mets)

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30
Q

Pediatric CNS tumors- Medulloblastoma - treatment - XRT -4

A

wonderful addition, but only if you are at least 3 years of age, IDEALLY OVER 5 YO

Attempting to use chemo to decrease dose of XRT or avoid it altogether.

inability to give XRT to younger kids hurts their chances of survival

Some low risk patients can be cured with post-op radiation

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31
Q

Pediatric CNS tumors- Medulloblastoma - treatment - chemo -5

IS THERE A SOC?

A

NO SOC

Cisplatin, vincristine, prednisone, cyclophosphamide, lomustine and topotecan have been used post-op.

Chemo used to avoid XRT in very young (<3)

Chemo can be used with XRT to decrease the dose of XRT used in low risk patients

Unclear if chemo improves survival in low risk patients

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32
Q

Pediatric CNS tumors- Medulloblastoma - treatment - chemo examples -2

A

CVP

lomustine (CCNU) 100mg/m2 po x 1
vincristine 1.5mg/m2 IV weekly x 3
prednisone 40mg/m2 po x 14 days

Cisplatin 90mg/m2 IV x 1
Lomustine 100mg/m2 PO x 1
Vincristine 1.5mg/m2 weekly x 3

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33
Q

Most important prognostic factors for medulloblastoma -3

A

metastatic stage M

adjuvant treatment

residual tumor

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34
Q

Pediatric CNS tumors- Medulloblastoma - prognosis -7

A

60-80% survival rate based on how aggressive surgery was as well as chemo, XRT

Poor risk: 
less than 3 years of age, 
subarachnoid seeding, 
greater than 1.5cm2 tumor remaining post-op, 
histology, 
diploid DNA content, 
C-myc amplification, 
deletion of 17p
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35
Q

Pediatric CNS tumors- Astrocytomas - background

NOT COVERED IN VIDEO

A 
#Most are slow growing (low grade): I and II
#About 20% are malignant (anaplastic astrocytoma or glioblastoma)
# 2 histologic types:  juvenile pilocytic astrocytomas and diffuse astrocytomas
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36
Q

Pediatric CNS tumors- Astrocytomas - signs and sx

A

associated with increased ICP, seizures, visual disturbances, can be similar to medulloblastoma depending on their location

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37
Q

Pediatric CNS tumors- Astrocytomas - treatment - surgery

A 
#curative intent for Cerebellar astrocytomas 
# malignant astrocytomas and glioblastoma, the goal is maximal resection
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38
Q

Pediatric CNS tumors- Astrocytomas - tx - XRT

A 
# avoid in kids <3-5 if possible
#prolong time to tumor progression if there was an incomplete resection of malignant astrocytoma or glioblastoma
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39
Q

Pediatric CNS tumors- Astrocytomas - tx - chemo

A 
#multiagent, dose-intense
#cyclophosphamide, cisplatin, carboplatin, etoposide, vincristine, carmustine (carboplatin is in most regimens since it is among the most active)  
#Chemo used where radiation deferred or failed or where surgery not a good option
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40
Q

Pediatric CNS tumors- Astrocytomas - tx - chemo example regimen

A

Carboplatin 175mg/m2 IV weekly x 4

Vincristine 1.5mg/m2 (0.05mg/kg if less than 12kg) IV weekly

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41
Q

Pediatric CNS tumors- Astrocytomas - prognosis

A 
#Worst prognosis: malignant astrocytoma and glioblastoma
#Extent of resection important for cerebellar gliomas
#The older the patient, the worse the prognosis
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42
Q

Pediatric CNS tumors- Brain stem gliomas - background

NOT COVERED IN VIDEO

A 
#Most frequently occur between 5-10 yo
#Usually in pons, can be in medulla and midbrain
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43
Q

Pediatric CNS tumors- Brain stem gliomas - signs and sx

A

rarely cause increased ICP until late in course
Extra ocular muscle paresis and diplopia, facial weakness, cranial nerve dysfunction, personality changes
Spastic gait, hemiparesis

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44
Q

Pediatric CNS tumors- Brain stem gliomas - staging and prognosis

A 
#diffuse infiltrative (usually in pons, poor prognosis, median OS <1yr)
#focal (in medulla or midbrain) - good prognosis if can be removed surgically +/- radiation – survival 50-100%
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45
Q

Pediatric CNS tumors- Brain stem gliomas - tx - surgery

A

remove as much as possible while preserving neurologic function

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46
Q

Pediatric CNS tumors- Brain stem gliomas - tx - XRT

A

Mainstay of treatment for diffuse infiltrative, also for palliation and to control neurologic symptoms

47
Q

neuroblastoma - background and incidence -4

A

Only 650 cases/year in US

36% occur before 1 year of age

90% occur before 5 years of age

Median age at diagnosis is 22 months old

48
Q

neuroblastoma - pathophysiology -5

A

Arise in the adrenal medulla or paraspinal sites of sympathetic nervous system tissue (anywhere along the sympathetic tract)

49
Q

neuroblastoma - risk stratification -6

A

40% of kids have low to intermediate risk dz which is cured by surgery or surgery plus chemo

Based on age, stage, and tumor biology

50
Q

neuroblastoma - prognosis -3

A

5 yr OS for infants is about 90% and about 55% for older children

Age 95%

5 yr DFS for kids with Stage 4 is about 35%

51
Q

neuroblastoma -Positive prognostic factors (8pts)

A

hyperdiploidy

normal n-myc copy number (THIS IS INTRINSIC TO DZ)

low LDH

low ferritin

high VMA/HVA ratio

neuron-specific enolase <100ng/ml

DNA CONTENT: HYPERDIPLOID (POOR= DIPLOID)

expression of HA-ras p21 or TRK gene

52
Q

neuroblastoma - diagnosis -3

A

Urine catecholamines vanillylmandelic acid (VMA) and homovanillic acid (HVA) are elevated in at least 85% of patients. The higher the VMA:HVA ratio, the better the prognosis.

Neuron specific enolase elevated in 95% pts with widespread disease.

Ferritin is produced by neuroblastoma cells and is elevated in 50% of patients with Stage III and IV disease.

53
Q

neuroblastoma - staging -3

A

Stage 1-4, plus 4S (which doesn’t fit the scheme of higher number = worse prognosis)

4S is localized primary tumor with dissemination limited to skin, liver and/or bone marrow (limited to infants less than 1 year of age) Usually hyperdiploid without N-myc amplification. GOOD OUTCOME.

Mostly stage 3 or 4

54
Q

neuroblastoma - tx - surgery -1

A

resection can cure localized disease

55
Q

neuroblastoma - tx - XRT -1

A

not useful for disseminated disease even though neuroblastoma is radiosensitive

56
Q

neuroblastoma - tx -chemo -2

MOST AGRESSIVE DOSING OF ALL PEDS ONC

A

Used in all but stage 1 and 4S disease

Cyclophosphamide, ifosfamide, doxorubicin, cisplatin, carboplatin, vincristine and epipodophyllotoxins have been used.

57
Q

neuroblastoma - tx -Biologic response modifiers (4pts)

MAY DOUBLE OS OUTCOMES FROM 30 TO 66% - SO FAR EARLY DATA

A

Differentiating agents (retinoic acid): used after stem cell transplant

mIBG to target radiotherapy for metastatic neuroblastoma

anti-GD2 ganglioside = ch14.18 monoclonal antibody USE AFTER TRANSPLANTATION (TX CAUSES A LOT OF PAIN - STIMULATES NERVES - USE OPIOIDS)

USE retinoic acid + anti-GD2 ganglioside AFTER STEM CELL TX to improve OS

58
Q

neuroblastoma - tx - stage 1 -2

THINK AGE AND STAGE +N-MYC

A

Surgery cures > 90%.

N-myc positive requires chemo

59
Q

neuroblastoma - tx -Stage 2A, infant 2B and 3 -5

THINK AGE AND STAGE +N-MYC

A

surgery

Chemo if N-myc amplified or recurrence.

Chemo includes cyclophosphamide and doxorubicin. Failures get cisplatin and etoposide.

Can use XRT for residual disease after second look surgery

cyclophosphamide 150mg/m2/day IV x 7 days
doxorubicin 35mg/m2 IV on day 8
Repeat q3 wks x 5

60
Q

neuroblastoma - tx - Stage 2B, 3 (tumors with positive nodes) -3

THINK AGE AND STAGE +N-MYC

A

50-70% cure rate with intensive therapy.

Surgery, chemo, and possible radiation

Ex:
vincristine 1.5mg/m2 IV on day 1
cisplatin 80mg/m2 IV over 24 hours on day 1
etoposide 200mg/m2 IV over 4 hours on day 3
alternate with
vincristine 1.5mg/m2 IV on day 1
cyclophosphamide 600mg/m2 IV on day 1
etoposide 200mg/m2 IV over 4 hours on day 1
carboplatin 500mg/m2 IV on day 1

61
Q

neuroblastoma - tx - Stage IV - concepts -4

THINK AGE AND STAGE +N-MYC

A

Least responsive to chemo

Survival depends strongly on age, infants respond to less aggressive courses than older kids

Even with aggressive treatment, OS usually no better than 20%

High risk = Stage 4, and stage 2,3,4S with N-myc amplification

62
Q

neuroblastoma - tx - Stage IV - tx plan -3

THINK AGE AND STAGE +N-MYC

A

Aggressive induction chemo

Consolidation with intensive chemo OR high dose chemo, TBI, and HSCT

SCT can be allo if there is a matched sibling, or auto

63
Q

neuroblastoma - tx - Stage IV - chemo regimen -2

KNOW
VINCRISTINE MAX 2MG OVER 72H

KNOW HOLD PARAMETERS

A

cyclophos 2.1gm/m2/day with mesna on days 1 and 2 (HUGE DOSE)
doxorubicin 25mg/m2 on days 1,2, and 3
vincristine 0.67mg/m2/day as bolus or continuous infusion on days 1, 2, and 3 (max of 2mg over 72 hours)
ALTERNATE with
Cisplatin 50mg/m2/day on days 1 to 4
etoposide 200mg/m2/day on days 1 to 3

HOLD TX IF ANC <100K

64
Q

neuroblastoma - tx - Stage IV - biologic response modifiers -3

A

Retinoic acid 160mg/m2/day x 14 days for 4 courses (helps neuroblastoma cells differentiate)

Monoclonal antibodies to neuroblastoma antigens

USE AFTER HSCT

65
Q

neuroblastoma - tx - Stage 4S - concepts -2

A

Surgical resection will cure most

Clinical course depends on age and liver involvement at diagnosis. If N-myc amplification, treat as high risk

66
Q

wilms tumor -5

A 
#Due to loss of function in tumor suppressor genes
~500 cases/year in US

Peaks at 2-3 years of age, rare after 6 years
sporadic and familial types

Familial types tend to be bilateral and occur at younger age (1-2% of cases)

Usually detected by caregiver who notices an abdominal mass

25% have hypertension

67
Q

Wilms tumor - pathophysiology -6

A

Favorable histopathology (85% of cases): well differentiated and resembles developing embryonic tissue

Unfavorable histopathology: anaplastic, clear cell, rhabdoid

Local spread – infiltrated renal capsule, involvement of renal sinus, tumor in intrarenal vessels

Distant spread to: lungs, regional lymph nodes and liver

Neuroblastoma displaces the kidney but Wilm’s distorts the kidney

Use ultrasound to check for thrombus in renal veins and inferior vena cava. Stage is increased if thrombus attached to vessel walls

68
Q

Wilms tumor - staging -5

A

I = limited to kidney, complete excision, intact capsule, not ruptures, no residual

II = extends beyond kidney, but completely excised. Lymph nodes negative

III = Residual tumor present

IV = hematogenous mets or lymph node mets outside abdomen

V = bilateral disease

69
Q

Wilms tumor - surgery -3

A

most important, ALL STAGES

check both kidneys,

can use chemo first to shrink tumor or if extensive vena cava involvement

70
Q

Wilms tumor - XRT -3

A

if nodes involved or metastatic disease,

peritoneal seeding,

spillage during surgery or rupture (No abdominal exams)

2 DRUGS ARE RADIOSENSITIZERS - THESE DRUGS ARE HELD DURING CHEM

71
Q

Wilms tumor - chemo -3

A

vincristine, dactinomycin, doxorubicin, and cyclophosphamide are the most active

1m2 kid = 30kg

For kids less than 1yo, doses are cut in half after converted to mg/kg to reduce toxicity - KEY

72
Q

Wilms tumor - chemo by stage - I and II (favorable) -2

TREATMENT IS BY STAGE AND RISK STRATIFICATION (HISTOLOGY)

FOR VINCRISTINE NEED TO BE ON A GOOD BOWEL REGIMEN AND WATCH NEUROTOX

DACTINOMYCIN IS HIGHLY EMETOGENIC

A

Dactinomycin 0.045mg/kg/dose (1.35mg/m2 for patients > 30kg (max 2.3mg) start within 5 days of nephrectomy during week 0 and then week 3,6,9,12,15,18

73
Q

Wilms tumor -chemo by stage -Stage III + IV and stage II-IV focal anaplasia (favorable histology) -4

HOLD DACTINOMYCIN AND DOXORUBICIN DURING XRT

TREATMENT IS BY STAGE AND RISK STRATIFICATION (HISTOLOGY)

A

Dactinomycin 0.045mg/kg IV (max 2.3mg) within 5 days of nephrectomy during week 0, then on weeks 6, 12, 18, and 24

74
Q

Wilms tumor - chemo by stage - Stage II to IV, stage I-IV clear cell, and stage II-IV diffuse anaplasia, unfavorable -5

HOLD DACTINOMYCIN AND DOXORUBICIN DURING XRT

TREATMENT IS BY STAGE AND RISK STRATIFICATION (HISTOLOGY)

A

Surgery and post op radiation to tumor bed

75
Q

Wilms tumor - prognosis -5

A

Based on stage and histology

76
Q

Rhabdosarcoma - background -4

A

Can occur anywhere, usual sites are GU, extremity, head and neck, trunk, orbit and retroperitoneum, prostate

77
Q

Rhabdosarcoma - natural history -2

A

Spreads locally and via lymph and blood.

Primary sites of metastatis are regional lymph nodes, lungs, bone and bone marrow (lung is most common)

78
Q

Rhabdosarcoma - surgery -1

A

if tumor is resectable

only 1/3 resectable

all will relapse with surgery alone

79
Q

Rhabdosarcoma - XRT -1

A

for local control (have to hold dactinomycin for radiation)

routine for higher stages =group 2-4

80
Q

Rhabdosarcoma - chemo -5

IRS clinical grouping system - based on how much tumor you clear with surgery

A

most relapse without chemo - given by group

81
Q

Rhabdosarcoma - VAC chemo -3

PAY ATTENTION TO MAX DOSES

A

vincristine 1.5mg/m2 IV weekly

dactinomycin 1.5mg/m2 or 0.045mg/kg (2.5mg max) IV every 3 weeks. (Using mg/kg due to increased risk of VOD in younger kids)

cyclophosphamide 1.2 or 2.2 gm/m2 IV every 3 weeks (using 1.2gm/m2 currently to try to decrease toxicity (sterility) without affecting survival rates)

82
Q

Rhabdosarcoma - prognosis -5

A

Overall 70% long term survival

Most important variable is clinical group or extent of disease at diagnosis, second most important is primary site.

Histology is also of prognostic significance. Best to worst: Botryoid, embryonal, alveolar, undifferentiated

Without complete tumor obliteration, cure is not possible.

HSCT doesn’t improve survival in high risk patients

83
Q

Rhabdosarcoma - late effects of chemo -6

A

Hemorrhagic cystitis

Infertility

Cardiomyopathy

Adhesions

Secondary malignancy (ex. radiation-related sarcomas and secondary AML

Renal Fanconi’s syndrome with ifosfamide

84
Q

retinoblastoma - background -5

A

300 cases in US each year

75% of cases are unilateral (ONLY GERM MUTATIONS CAUSE BILATERAL)

Majority in very young children 95% before 5 years and 66% before 2 years

The RB gene is a tumor suppressor gene and it gets inactivated

2 Types: hereditary (40%) and sporadic (60%)

85
Q

retinoblastoma - signs and sx -3

A

Leukokoria (lack of red reflex in eye) – white pupil

Strabismus (having a squint)

Red/painful eye

86
Q

retinoblastoma - screening -2

A

Kids with a positive family history have full eye exams shortly after birth and at 6 weeks of age and every 2-3 months until 2 years old.

This is one of the very few pediatric tumors that can be screened.

87
Q

retinoblastoma - natural hx -4

A

Arises in retina

Can extend locally or spread distantly

Local spread = growth in intraocular space

Mets occur when tumor grows along optic nerve, spread to CNS or spread via lymph or blood

88
Q

retinoblastoma - dx and staging -4

A

Eye exam with ophthalmoscope
Ultrasound
CT

Staging based on number and size of tumors and whether or not there is vitreous seeding

89
Q

retinoblastoma - surgery -2

A

enucleation for advanced disease (not done as much as in the past).

Can use laser photocoagulation or cryotherapy if the tumor is small enough

90
Q

retinoblastoma - XRT -2

A

to try to control the disease and retain the eye and useful vision.

increased risk of second cancers (OSTEOSARCOMA)

91
Q

retinoblastoma - chemo -6

A

IV and or IT chemo if metastatic disease present.

Can use it to minimize need for XRT or enucleation.

Vincristine 1.5mg/m2 (0.05mg/kg if less than 36 mo) IV x 1

Etoposide 150mg/m2 (5mg/kg if less than 36mo) IV daily x 2

Carboplatin 560mg/m2 (18.6mg/kg if less than 36mo) x 1

Combination chemo and radiation is used for stage IV and V

92
Q

retinoblastoma - prognosis -2

A

90% survive retinoblastoma, but patients with germinal mutation tend to get second non-ocular malignancy (osteogenic sarcoma)

Retention of vision depends on the size of the tumor and its location

93
Q

osteosarcoma - background -4

A

Primarily seen in adolescents (during growth spurt)

A high-grade sarcoma

Usually occurs in distal femur, proximal tibia, or proximal humerus. Most common is distal femur

Can involve flat bones (ex pelvis)

94
Q

osteosarcoma - risk factors (4pts)

A

Bone diseases (osteogenesis imperfecta and Paget’s disease) predispose elderly to osteosarcoma

Ionizing radiation

Genetic predisposition (ex retinoblastoma and risk of secondary osteosarcoma)

Alkylating agents

95
Q

osteosarcoma - signs and sx -3

A

Pain/mass/swelling over the involved area

About 20-25% present with metastatic disease.

Most common site is the lungs

96
Q

osteosarcoma - dx w/u -4

A

x-rays

CT/MRI

Biopsy

Can see increased alkaline phosphatase and LDH

97
Q

osteosarcoma - staging

A

Stage 1 (low grade)

Stage II (high grade) – most are high grade

Stage III (distant mets)

A OR B is if extramedullary (broken out of bone)

98
Q

osteosarcoma - surgery -4

NEED CLEAN MARGINS FOR LONG TERM OS

CAN YOU USE ALONE?

A

must remove the primary tumor for local control.

Limb salvage or amputation

Metastatic disease must be completely removed in order to get a cure

need chemo also otherwise 80% recurrence

99
Q

osteosarcoma - XRT

INCLUDE OR NOT?

A

Osteosarcoma is not radiosensitive, so radiation is not used this is a difference vs Ewing’s sarcoma

100
Q

osteosarcoma - chemo principles -3

A

relatively drug-resistant due to p-glycoprotein

Few drugs are effective

High dose methotrexate alternating with doxorubicin and cisplatin - NEED CLOSE MONITORING OF RENAL FUNCTION AND MTX LEVELS

101
Q

osteosarcoma - chemo regimen -3

GIVE TO ALL CYCLES

IF RENAL TOX - WORK UP PRIOR TO CHEMO

A

Cisplatin 120mg/m2 IV over 4 hrs on day 1

Doxorubicin 25mg/m2/day on days 1, 2, and 3 as a continuous infusion

Alternate with methotrexate 12gm/m2 IV over 4 hours on days 21 and 28 with leucovorin starting 24 hours from the start of methotrexate. (MAX methotrexate dose is 20 grams)

102
Q

osteosarcoma - neoadj chemo principles -4

A

Give chemo for a few cycles, then assess tumor response at time of resection.

Less than 90-95% tumor necrosis is associated with poor risk disease

Shrinking the tumor with chemo prior to surgery improves the chances of limb salvage instead of amputation

Overall, neoadjuvant chemo hasn’t improved outcome vs post-surgical chemo

103
Q

osteosarcoma - prognosis (6pts)

A

Surgery and adjuvant chemo have 50-75% disease-free survival at 2-5 years.

Overt metastatic disease has poor outcome

Axial primaries (pelvis) have worse outcomes

Age >20 is better, <10 is worse

Females have better outcome

Increased levels of alkaline phosphatase are associated with increased risk of metastatic disease and poor prognosis

104
Q

Ewing’s Sarcoma - background (6pts)

A

Also most commonly seen in adolescents

Considered a bone tumor, but can arise in soft tissue

Primitive neuroectodermal cell tumor, small round blue cell tumor (unique translocation is t(11;22)

Most frequent site is pelvis, then the femur, tibia, and humerus

Can arise in any bone

Osteosarcoma is usually in long bones in extremities, while Ewing’s is usually in the axial skeleton

105
Q

Ewing’s Sarcoma - signs and sx -3

A

Pain and swelling of the affected area

May have systemic signs and symptoms (unlike osteosarcoma), especially with metastatic disease: fatigue, anorexia, weight loss and fevers

Can be an extraosseous tumor (no bone involvement)

106
Q

Ewing’s Sarcoma - natural hx and staging- (unusual) (7pts)

A

Without systemic treatment, over 90% of patients develop mets, mostly in the lungs. Can also spread to other bones and the bone marrow

Highly malignant

No universally accepted staging system

Presence of metastatic disease is the important issue

Elevated LDH is associated with metastatic disease and a poor prognosis

Spread to lymph nodes is rare (as opposed to rhabdo, osteo and PNET which involve lymph nodes more commonly)

Mets to paraspinal area are common

107
Q

Ewing’s Sarcoma - surgery -6

WHAT DOES THIS DEPEND ON?

USUALLY DO SURGERY OR XRT WITH CHEMO

A

POSSIBILITY FOR COMPLETE RESECTION AND ANATOMY (QOL ISSUES, CHANCE FOR CURE)

Role is for local control

Can use surgery or radiation for local control

Neoadjuvant chemo is given to assess tumor responsiveness and treat micrometastatic disease.

Neoadjuvant chemo may improve resectability of tumor

Can do limb sparing or amputation

108
Q

Ewing’s Sarcoma - XRT -3

USUALLY DO SURGERY OR XRT WITH CHEMO

A

very radiosensitive.

Use for lesions that are difficult to remove surgically

can be used if surgery would result in loss of nerve function

109
Q

Ewing’s Sarcoma - chemo

VCR regimen alternating with IE

HOW DO YOU GIVE IN NON-METS DZ?

A

Vincristine 1.5mg/m2 IV
Doxorubicin 75mg/m2 IV over 48 hours
Cyclophosphamide 1200mg/m2 IV

Alternating with:
Etoposide 100mg/m2 IV daily x 5 days
Ifosfamide 1.8gm/m2 IV daily x 5 days

ONLY GIVE IE IN NON-METS DZ.

DO NOT HOLD FOR MYELOSUPPRESSION

110
Q

Ewing’s Sarcoma - HSCT

A

Patients with multifocal or metastatic disease at diagnosis have very poor prognosis

High dose chemo and HSCT is now front line tx

111
Q

Ewing’s Sarcoma - prognosis

DRIVEN LARGELY BY EXTENT OF DZ

A 
#Most important factor is extent of disease at diagnosis
#Increased LDH predicts metastatic disease and poor prognosis
#Mets to bone marrow or bone has a poor prognosis
#Multifocal disease at presentation has bad prognosis
#Patients less than 10yo have better prognosis than older patients
#Distal sites are more favorable 
#Unfavorable sites include pelvic or sacral lesions, intermediate sites include humerus, femur, and rib.  
#OS for distal extremity is 60-70% and pelvic is 35%
#Tumor volume  100ml
#Pathologic response to neoadjuvant chemo (good response is favorable prognostic sign)
112
Q

nuances in peds onc -7

A

no established screening tests

anthracycline - delayed cardio tox more common

cisplat - hydration 2x maintenance, correct CrCl for size

ifos - renal Fanconi’s = spill electolytes, mostly phos, and spill glucose (see at doses >70g/m2)

infant dosing - reduce or by kg (with BSA nomogram can overdose kids) ~30kg = 1m2 BSA

IT dosing - based on age (a dose in mg/m2 WOULD BE INCORRECT)

slow use of new agents in clinical trials despite high clinical trial enrollment (~85%)

113
Q

how much is ~30kg equal to in BSA?

A

1 m2

114
Q

How do you manage Fanconi’s? hypophos, glucose in urine

A

change ifos to cyclophos

BCOP (32 decks)

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