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Pediatrics > ROQ: RMS, NB, Wilms, Ewings, HL > Flashcards

ROQ: RMS, NB, Wilms, Ewings, HL Flashcards

1
Q

WIthin how much time after surgery should a Wilms tumor be treated?

A
  • 9 days!
    – Unf. histology: Delay beyond 10 days is a/w higher chance of abdominal recurrence
    – Fav. histology: Initiation within 14 days is acceptable
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2
Q

Within how much time after surgery should a RMS be treated?

A
  • RT sequencing depends on risk grouping
    – Low-risk: Week 13
    – Int-risk: Week 13
    – High-risk: Week 20
    – If RT does not start by week 20, start it by week 47
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3
Q

What radiation dose can be used to treat spinal cord compression 2/2 neuroblastoma?

A
  • < 3 yrs
    – 9 Gy (1.8 Gy x 5 fx)
  • > 3 yrs
    – 21.6 Gy

Note that CHT alone is preferred to spare children late effects of RT

Hook: 9 x 3 = 21

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4
Q

What percentage of neuroblastoma pts present w/ spinal cord compression?

A

10-15%

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5
Q

What is the TNM classification for RMS?

A
  • T:
    – T1: Confined to the anatomic site of origin
    — T1a: ≤ 5 cm
    — T1b: > 5 cm
    – T2: Extension beyond site of origin
    — T2a: ≤ 5 cm
    — T2b: > 5 cm
  • N: N0 or N1
  • M: M0 or M1
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6
Q

What is the staging for RMS?

A

Staging is based on size, location, nodal status, presence of metastases

  • Stage 1: Favorable sites regardless of size, invasiveness, or LN status (BONG)
    – Biliary?
    – Orbit
    – non-para meningeal H&N
    – GU, Non-bladder/prostate
  • Stages 2 and 3 are all non-favorable sites.
    – Stage 2: <5 cm that is LN-
    – Stage 3: <5 cm and LN+, or >5cm.
  • Stage 4: M1
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7
Q

What is the grouping for RMS?

A

Group: determined by the extent of surgical resection

  • Group 1: R0 resection
    – 1a: confined to the muscle or organ,
    – 1b: infiltration outside the muscle or organ
  • Group 2; R1 resection and/or LN+
    – 2a: R1 resection
    – 2b: LN+
    – 2c: R1 resection + LN+
  • Group 3: STR
    – 3a: bx only
    – 3b: >50% resected w/ leftover gross disease
  • Group 4 is any tumor with distant metastases.
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8
Q

What are the risk categories for RMS?

A

Risk Categories:

  • Low:
    – Stage 1 embryonal tumor
    – Stage 2/3 group 1/2 embryonal tumor (i.e. tumor in an unfavorable site s/p GTR with negative or microscopic margins+
  • Intermediate:
    – Stage 2/3 group 3 Embryonal tumor.
    – Any alveolar histology w/o metastases.
  • High:
    – All stage 4 and/or group 4 patients
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9
Q

What is the usual CHT and different RT doses for RMS?

A
  • All non-metastatic RMS receive VAC (reduced C dose for some)
    – Group IV receives VCRARIN x 6wks → VAC-IW q2wks (ARST0431)
  • Group and RT Dose:
    – I: (embryonal only): No RT
    – I: (alveolar only): 36 Gy
    – II LN-: 36 Gy
    – Il LN+: 41.4 Gy
    – Ill, orbits only w/ CR to CHT: 45 Gy
    – III, all others (including orbit w/ PR to CHT): 50.4 Gy
    – IV (primary site and mets): 50.4 Gy
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10
Q

What are the distinguishing features b/w Neuroblastoma and Wilms tumor?

A
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11
Q

What is the usual anatomic location of a neuroblastoma vs. a Wilms tumor?

A
  • Neuroblastoma: Suprarenal
  • Wilms tumor: Intrinsic renal
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12
Q

What is the COG staging for Wilms tumor?

A
  • 1: Limited to kidney, completely excised, no penetration of capsules
  • 2: Extends beyond the kidney but completely excised
  • 3: BS-SLURPP
    – Biopsy
    – Spillage (diffuse or local)
    – Subtotal resection/positive margin = gross or microscopic residual
    – Lymph node(s)
    – Unresectable (pre-operative chemotherapy)
    – Rupture
    – Peritoneal implants or penetration
    – Piecemeal or separate removal
  • 4: Hematogenous Mets (lung, liver, bone, brain, etc) or LNs outside abdominopelvis
  • 5: B/l Wilms’ tumor
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13
Q

What is the surgical staging for neuroblastoma?

A
  • 1: Localized, complete excision.
  • 2A: Localized, gross residual, negative lymph nodes.
  • 2B: Localized, with or without gross residual, positive ipsilateral lymph nodes. Contralateral lymph nodes negative.
  • 3: Unresectable, infiltrating across spine/midline or localized tumor with contralateral nodes.
  • 4: Dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs.
  • 4S: Localized tumor (stages 1, 2) with spread to skin, liver, and/or marrow (<10%) in an infant
    < 1 year-old.
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14
Q

What is the risk grouping for neuroblastoma?

A
  • L1: localized tumor not involving vital locations as defined by imaging features and involving
    only one organ system
  • L2: locoregional tumor with the presence of one or more imaging-defined risk factors
  • M: metastatic disease
  • MS: metastatic with metastases limited to skin, liver, and/or bone marrow in patients less than 18 months
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15
Q

What are the indications for WART for Wilms tumor?

A
  • Any stage III fav. histology Wilms
  • SPAR
    – Spillage
    – Peritoneal Implants
    – Ascites
    – Rupture
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16
Q

What is the 5 yr OS for osteosarcoma tx w/ surgery alone?

A

~20%

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17
Q

What is the 5 yr OS for osteosarcoma tx w/ neoadj. CHT → surgery → adj. CHT x4-6 mos?

A

60-70%

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18
Q

What are fav. and unfav. sites for RMS?

A
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19
Q

What are the distinguishing features of neuroblastoma?

A
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20
Q

Why is neuroblastoma MIBG+?

A

MIBG concentrates in adrenergic tissue, which is the progenitor of neuroblastoma.

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21
Q

Should neuroblastoma be biopsied to confirm dx?

A

yes!

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22
Q

Should wilms tumor be biopsied to confirm dx?

A
  • No!
  • Avoid any Bx slip-ups!
  • Upfront surgical resection is the SOC
  • Except for stage V (b/l Wilms)
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23
Q

What is the general tx of Ewing’s sarcoma?

A
  • Ind. CHT (Wks 1-12) with Vincristine, Adriamycin, and Cyclophosphamide alternating with Ifosfamide and Etoposide (VAC-IE)
  • Local Therapy (Week 12) with surgery, RT, or surgery + RT (Adriamycin (and actinomycin-D if given) are held during RT)
  • Adj. CHT (up to 48 weeks)

Memory Hook: Slighlty akin to the RMS tx paradigm!

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24
Q

What is the 5-yr local failure rate of pelvic Ewing sarcoma tx w/ induction CHT f/b RT (w/o surgery)?

A

20-30%

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25
Q

What is the 5-yr local failure rate of pelvic Ewing sarcoma tx w/ induction CHT f/b surgery (w/o RT)?

A

~13%

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26
Q

What is the 5-yr local failure rate of pelvic Ewing sarcoma tx w/ induction CHT f/b surgery w/ RT?

A

0%

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27
Q

What is the 5-yr local failure rate of Ewing’s sarcoma of the extremities tx w/ induction CHT f/b RT (w/o surgery)?

A

~15%

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28
Q

What is the 5-yr local failure rate of Ewing’s sarcoma of the extremities tx w/ induction CHT f/b surgery + RT?

A
  • 5.4%
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29
Q

What is the 5-yr local failure rate of Ewing’s sarcoma of the extremities tx w/ induction CHT f/b surgery w/o RT?

A

~4%

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30
Q

What is the 5-yr local failure rate of Ewing’s sarcoma in children < 18 yrs vs. > 18 yrs?

A

7% vs. 12% (p=0.02)

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31
Q

Where do the majority of the Ewing sarcoma pts fail, locally or distantly?

A

Distally

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32
Q

What is the general RT dose for whole lung irradiation for unfav. and fav. histology Wilm’s tumor?

A
  • 12 Gy in 8 fx
    – 1.5 Gy / fx
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33
Q

When is lung RT given in Wilms tumor?

A
  • Unfav histology w/ lung mets → RT
  • Fav histology w/ lung mets → chemo → inadequate response → RT
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34
Q

What is the general RT dose for whole lung irradiation for an Ewing sarcoma?

A
  • Whole Lung: 12-15 Gy
  • Pleural cavity: 15-18 Gy
  • Lesions: 40-50 Gy
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35
Q

What are the PORT doses for Wilms tumor?

A
  • 10.8 Gy
    – Stage I-III w/ fav. histology w/ focal anaplasia
    – Stage III w/ fav. histology
  • 19.8 Gy
    – Stage III w/ diffuse anaplasia
    – Rhabdoid tumor of the kidney (RTK), all stages
    – Unresected LN mets
    – Whole liver radiation (met disease)
  • 21.3 Gy:
    – Residual disease that measures > 3 cm (10.5 Gy whole abdomen + 10.8 Gy boost)

Note that the whole abdomen dose in 10.5 Gy, not 10.8 Gy!

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36
Q

What is the most common extracranial malignancy in children?

A

Neuroblastoma: 7-10%

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37
Q

What is high-risk neuroblastoma?

A
  • MYCN amplification
  • M stage (distant metastatic disease) AND older than 18 months
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38
Q

What is the general tx paradigm for high-risk neuroblastoma?

A

In order:
- - Induction chemotherapy
- Surgical resection
- Myeloablative chemotherapy f/b
- Tandem stem cell transplant
– 2 ASCT autologous transplants done ≤ s6 mos apart
- Radiotherapy
- Immunotherapy
- Isotretinoin.

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39
Q

What is the general tx paradigm for high-grade osteosarcoma?

A
  • NAC → surgery
  • R1 or R2 surgery → PORT
40
Q

What is the general tx paradigm for unresectable osteosarcoma?

A
  • NAC
    – Allows for shrinkage and smaller RT fields
  • RT to 60-70 Gy ± sensitizers
  • Adj. CHT
41
Q

What is the 3-yr EFS and OS for high-risk neuroblastoma receiving single vs. tabden ASCT?

A
  • Tandem vs. single
    – 3-yr EFS: 61.4 vs. 48.4 (p=0.008)
    – OS: No difference
42
Q

What is the general tx paradigm for low- and intermediate-risk neuroblastoma?

A
  • Low
    – Surgery ONLY
  • Intermediate:
    – Induction CHT → Surgery
43
Q

What was the pt population, randomization, and primary endpoint of AWS 0031 for Ewing Sarcoma?

A
  • Pts: Localized Ewing Sarcoma
  • Randomization: VDC (vincristine, doxorubicin, cyclophosphamide) alternating w/ lE (ifosfamide and etoposide)
    – q21 days (standard timing chemotherapy [STC])
    – q14 days (interval-compressed chemotherapy [IC])
  • Primary-directed therapy with surgery, RT, or both occurred on week 13
  • Primary Endpoint: EFS

Hook: 3 > 1 (compressed)

44
Q

What were the results of AWS 0031 for Ewing Sarcoma?

A

STC vs. IC:
- 5-yr EFS: 65% vs. 73% (p=0.0048)
– decreased rate of distant mets
- 10-yr EFS: 61% vs. 70% (p=0.03)
- 10-yr OS: 69% vs. 76% (p=0.040)
- No diff. in 10-yr incidence of second cancers

45
Q

When is RT indicated for a post-op Ewings sarcoma?

A
  • Positive margins
  • Residual disease
46
Q

What RT doses are used for Ewing sarcoma?

A
  • RT for Ewing’s
    – Primary: 50.4-55.8 Gy
    — Higher doses for def. tx
    — Lower doses for microscopic dz. + dz. near the cord
47
Q

How do you construct RT volumes for an Ewing sarcoma pt?

A
  • CTV45: Pre-chemo dz + 2 cm
  • CTV50.4: Pre-chemo bone + Post-chemo soft tissue dz
48
Q
A
49
Q

What RT doses are used for neuroblastoma?

A
  • Pre-op GTV: 21.6 Gy in 12 fx
  • Post-op residual gross disease: Boost to 36 Gy reasonable

Hook: Doses similar to Wilms

50
Q

What is the standard RT fx size for pediatric pts?

A
  • 1.5-1.8 Gy
51
Q

What is an appropriate palliative whole liver RT dose for pts w/ diffuse liver mets impairing respiration from metastatic neuroblastoma?

A

4.5 Gy in 3 fx

52
Q

What is an appropriate palliative whole liver RT dose for pts w/ diffuse liver disease from metastatic Wilms tumor?

A
  • < 12 mos → 10.8 Gy
  • ≥ 12 mos → 19.8 Gy
53
Q

What are some of the locations and tx goals of Ewing sarcoma?

A
54
Q

What is the most common age of presentation for Ewing sarcoma?

A

10-14 yrs

Hook: During the growth spurt

55
Q

Which sex and race are more predisposed to Ewing sarcoma?

A
  • Male > Females
  • Caucasians > Other races
56
Q

Do the majority of Ewing sarcoma pts present w/ localized or metastatic disease?

A
  • 75-80% present w/ localized disease
  • 20-25% w/ metastatic disease
57
Q

What is the 5-year OS for patients w/ localized Ewing sarcoma?

A

70%

58
Q

What is the 5-year OS for Ewing sarcoma patients w/ a solitary lung nodule?

A

~ 50%

59
Q

What is the 5-year OS for patients w/ metastatic Ewing sarcoma?

A

< 30%

60
Q

Which Ewing sarcoma anatomic sites should be considered for def. RT?

A
  • Inoperable Sites
    – Proximal locations
    – Pelvis
    – Vertebral body
  • You do not want to resect weight-bearing bones, DUH!
61
Q

Which Ewing sarcoma anatomic sites should be considered for surgery only?

A
  • Expendable sites
    – Proximal fibula
    – Lateral 4/5th of the clavicle
    – Parts of the ilium
62
Q

Which syndromes are a/w Wilms tumor?

A
  • WAGR: Mutation in the WT1 gene on ch 11
    – Wilms’ tumor
    – Aniridia
    – GU anomalies
    – mental Retardation.
  • Denys-Drash: Mutation in the WT1 gene on ch 11
    – Pseudohermaphroditism
    – Masangial renal sclerosis
    – Wilms’ tumor
  • Beckwith-Wiedemann
    – Macroglossia
    – Macrosomia
    – Midline abdominal wall defects
    – Ear creases/pits
    – Neonatal hypoglycemia
    – hemihypertrophy

##

Memory Hook:

Denys-Drash → Draq Queen sounding name → pseudohermaphrodism

63
Q

What are the standard borders of an abdominal RT field for Wilms tumor?

A
  • AP/PA fields
    – Sup: diaphragm
    – Inf: Bottom of obturator foramen
    – Block femoral heads
64
Q

What is the general tx paradigm for a Wilms tumor?

A
  • COG:
    – Upfront resection
    – CHT ± RT
  • SIOP:
    – Neoadj. CHT
    – Resection
65
Q

Which histologies are unfav. in Wilms tumor?

A
  • Unf:
    – Anaplasia (dense nuclear chromatin, nuclear pleomorphism, mitotic figures)
    – Clear cell sarcoma of the kidney
    – Rhabdoid tumor of the kidney.
66
Q

What % of pts present w/ fav histology Wilms tumor?

A
  • Fav: 90%
    – 2-yr mortality: 7.1%
  • Unfav: 10%
    – 2-yr mortality: 44%
67
Q

What is the EFS and OS benefit for Wilms tumor pt w/ lung nodules seen on CT but not CXR?

A
  • WT studies 4 and 5:
    – CT-only nodules tx w/ lung radiation vs. no RT
    — ~10% benefit in EFS and OS (NS)
    — EFS (82% vs. 72%; p = 0.13)
    — OS (91% vs. 83%; p = 0.46)
68
Q

For a Wilms tumor, what is the % bx rate of lung nodules identified on a CT scan?

A
  • Overall: ~75%
  • Isolated lung lesion: ~82%
  • Multiple lung lesions: ~69%
69
Q

What is the brain RT doses for a Wilms tumor pt w/ brain metastases?

A
  • Age ≥ 16 yrs
    – WBRT 30.6 Gy w/o boost
  • Age < 16 yrs
    – 21.6 Gy WBRT f/b 10.8 Gy conformal boost (32.4 Gy total)
70
Q

What % of RMS are embryonal?

A

60-70%

71
Q

What % of RMS are alveolar?

A

20-30%

72
Q

How does the prognosis of RMS relate to histology?

A
  • Favorable prognosis
    – Boytroid and Spindle Cell
  • Intermediate prognosis
    – Embryonal
  • Worst prognosis
    – Alveolar
73
Q

What are the pathognomonic findings of Ewing sarcoma on plain film?

A
  • Onion skinning
    – aka Moth- eaten
    – aka Permeative
74
Q

What are the pathognomonic findings of Osteosarcoma sarcoma on plain film?

A

Sunburst pattern

75
Q

What are the typical locations of Ewing sarcoma?

A

The diaphysis of long bones

76
Q

What are the typical locations of Ewing sarcoma?

A

Metaphysis (near growth plates) of long bones

77
Q

Which mutations are a/w embryonal RMS of the GU tract?

A

DICER1

78
Q

Which mutations are a/w alveolar RMS?

A

PAX3-and PAX7-FOXO1 gene fusions from t(2;13) or t(1;13)

79
Q

Which mutations are a/w Ewing’s sarcoma?

A

EWSR1 gene rearrangements t(11:22)

80
Q

When should LN dissection be performed in boys w/para testicular RMS?

A
  • < 10 yrs old → thin cut CT f/b workup of any suspicious nodes
  • ≥ 10 yrs old → RPLND (may harbor subclinical disease even w/ a -ve CT scan)
81
Q

What dose of RT is appropriate for children w/ painful bony metastases?

A
  • < 16 yrs old → 25.2 Gy to tumor + 1 cm
  • ≥ 16 yrs old → 30.6 Gy to the whole bone
82
Q

What is an ASKIN tumor?

A

PNET or Ewing sarcoma of the chest wall

83
Q

What is the 5-yr OS for patients w/ localized vs. metastatic osteosarcoma?

A
  • Localized: 75%
  • Metastatic: 25%
84
Q

How are RT volumes defined for Ewing sarcoma per modern protocols?

A
  • GTV1: pre-surg and pre-CHT disease
    – CTV1
    – GTV1 + 1 cm
    – Include regional LN chains for LN+
    – Edit at anatomic barriers
    – PTV1 = CTV1 + 0.5 cm
  • GTV2: residual disease after induction CHT ± surgery
    – CTV2 = GTV2 + 1 cm
    – PTV2 = CTV2 + 0.5 cm
  • Special considerations:
    – CW tumors w/ ipsilateral pleural nodules and/or isolated pleural fluid involvement:
    – CTV3: ipsilateral hemithorax.
    – PTV3 accounts for organ motion as well as a 0.5-1 cm geometric expansion
    – Vertebral body tumors: CTV1 to include the entire vertebral body.
    – Extremity tumors: circumferential irradiation of extremity lymphatics and treatment across a joint should be avoided unless absolutely necessary for tumor coverage.
    – Orbit: CTV should not extend outside of the bony orbit, providing there is no bone erosion of the orbit
    – Intra-abdominal/retroperitoneal/pelvic: whole abdominal radiotherapy is indicated for malignant ascites or diffuse peritoneal involvement
85
Q

How does N-MYC mutation affect EFS in neuroblastoma pts?

A
  • +N-MYC → 3-yr EFR 10%
  • -N-MYC → 3-yr EFR 93%
86
Q

How is focal anaplasia defined for Wilms tumor?

A

Anaplasia is confined to multiple clearly defined loci within the primary tumor

87
Q

When is a bone marrow biopsy (BMBx) required for RMS?

A
  • For all diagnoses
  • Usually required of all non-CNS peds malignancies besides CCSK, and Wilms tumor
88
Q

When is a brain MRI obtained for RMS?

A

RMS of Parameninges

89
Q

When is an LP done for RMS?

A

For suspected CNS involvement

90
Q

What are the 5-ry EFS for low, intermediate, and high-risk RMS?

A
  • Low: 85-90%
  • Int: 73%
  • High: 32%
91
Q

Which sites are considered parameningeal for RMS?

A
  • MMNNOOPP
    – Middle ear
    – Mastoid region
    – Nasal cavity
    – Nasopharynx
    – Pterygopalatine fOssa
    – Infratemporal fOssa
    – Paranasal sinus
    – Parapharyngeal region
92
Q

What are the 10-yr OS for favorable, and unfavorable histology Wilms tumors?

A
  • Favorable: Uniformly good prognosis
    – I: 97%
    – II: 93%
    – III: 90%
    – IV: 81%
    – V: 78%
  • Unfavorable
    – II-III: 50%
    – IV: 18%
  • Clear cell: 78%
  • Rhabdoid tumor of the kidney: 28%
93
Q

What % of newly dx Wilms tumor cases have a family hx of the disease?

A

1-2%

94
Q

What % of Wilms tumors are resectable at dx?

A
  • 90-95%, hence surgery w/o bx is the first plan of attack
95
Q

What are poor prognostic factors in Wilms tumor pts?

A
  • Focal/Diffuse anaplasia
  • Gain of 1q, loss of 1p, loss of 16q
96
Q

What is the median age of dx of NB?

A

22 mos

Pediatrics (3 decks)

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