Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

ROQs > [ROQs] PEDS, RMS, NB, Wilms, Ewings, HL > Flashcards

[ROQs] PEDS, 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A

10-15%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

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

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

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

A
  • Neuroblastoma: Suprarenal
  • Wilms tumor: Intrinsic renal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the indications for WART for Wilms tumor?

A
  • Any stage III fav. histology Wilms
  • SPAR
    – Spillage
    – Peritoneal Implants
    – Ascites
    – Rupture
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A

~20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

60-70%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are fav. and unfav. sites for RMS?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the distinguishing features of neuroblastoma?

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Why is neuroblastoma MIBG+?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Should neuroblastoma be biopsied to confirm dx?

A

yes!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What is the 5-yr local failure rate of **pelvic** Ewing sarcoma tx w/ induction CHT f/b surgery (w/o RT)?
~13%
26
What is the 5-yr local failure rate of **pelvic** Ewing sarcoma tx w/ induction CHT f/b surgery w/ RT?
0%
27
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)?
~15%
28
What is the 5-yr local failure rate of Ewing's sarcoma of the extremities tx w/ induction CHT f/b surgery + RT?
- 5.4%
29
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?
~4%
30
What is the 5-yr local failure rate of Ewing's sarcoma in children < 18 yrs vs. > 18 yrs?
7% vs. 12% (p=0.02)
31
Where do the majority of the Ewing sarcoma pts fail, locally or distantly?
Distally
32
What is the general RT dose for whole lung irradiation for unfav. and fav. histology Wilm's tumor?
- 12 Gy in 8 fx -- 1.5 Gy / fx
33
When is lung RT given in Wilms tumor?
- Unfav histology w/ lung mets → RT - Fav histology w/ lung mets → chemo → inadequate response → RT
34
What is the general RT dose for whole lung irradiation for an Ewing sarcoma?
- Whole Lung: 12-15 Gy - Pleural cavity: 15-18 Gy - Lesions: 40-50 Gy
35
What are the PORT doses for Wilms tumor?
- 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) ## Footnote Note that the whole abdomen dose in 10.5 Gy, not 10.8 Gy!
36
What is the most common extracranial malignancy in children?
Neuroblastoma: 7-10%
37
What is high-risk neuroblastoma?
- MYCN amplification - M stage (distant metastatic disease) AND older than 18 months
38
What is the general tx paradigm for high-risk neuroblastoma?
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.
39
What is the general tx paradigm for high-grade osteosarcoma?
- NAC → surgery - R1 or R2 surgery → PORT
40
What is the general tx paradigm for unresectable osteosarcoma?
- NAC -- Allows for shrinkage and smaller RT fields - RT to 60-70 Gy ± sensitizers - Adj. CHT
41
What is the 3-yr EFS and OS for high-risk neuroblastoma receiving single vs. tabden ASCT?
- Tandem vs. single -- 3-yr EFS: 61.4 vs. 48.4 (p=0.008) -- OS: No difference
42
What is the general tx paradigm for low- and intermediate-risk neuroblastoma?
- Low -- Surgery ONLY - Intermediate: -- Induction CHT → Surgery
43
What was the pt population, randomization, and primary endpoint of AWS 0031 for Ewing Sarcoma?
- 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 ## Footnote Hook: 3 > 1 (compressed)
44
What were the results of AWS 0031 for Ewing Sarcoma?
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
When is RT indicated for a post-op Ewings sarcoma?
- Positive margins - Residual disease
46
What RT doses are used for Ewing sarcoma?
- 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
How do you construct RT volumes for an Ewing sarcoma pt?
- CTV45: Pre-chemo dz + 2 cm - CTV50.4: Pre-chemo bone + Post-chemo soft tissue dz
48
49
What RT doses are used for neuroblastoma?
- Pre-op GTV: 21.6 Gy in 12 fx - Post-op residual gross disease: Boost to 36 Gy reasonable ## Footnote Hook: Doses similar to Wilms
50
What is the standard RT fx size for pediatric pts?
- 1.5-1.8 Gy
51
What is an appropriate palliative whole liver RT dose for pts w/ diffuse liver mets impairing respiration from metastatic neuroblastoma?
4.5 Gy in 3 fx
52
What is an appropriate palliative whole liver RT dose for pts w/ diffuse liver disease from metastatic Wilms tumor?
- < 12 mos → 10.8 Gy - ≥ 12 mos → 19.8 Gy
53
What are some of the locations and tx goals of Ewing sarcoma?
54
What is the most common age of presentation for Ewing sarcoma?
10-14 yrs ## Footnote Hook: During the growth spurt
55
Which sex and race are more predisposed to Ewing sarcoma?
- Male > Females - Caucasians > Other races
56
Do the majority of Ewing sarcoma pts present w/ localized or metastatic disease?
- 75-80% present w/ localized disease - 20-25% w/ metastatic disease
57
What is the 5-year OS for patients w/ localized Ewing sarcoma?
70%
58
What is the 5-year OS for Ewing sarcoma patients w/ a solitary lung nodule?
~ 50%
59
What is the 5-year OS for patients w/ metastatic Ewing sarcoma?
< 30%
60
Which Ewing sarcoma anatomic sites should be considered for def. RT?
- Inoperable Sites -- Proximal locations -- Pelvis -- Vertebral body - You do not want to resect weight-bearing bones, DUH!
61
Which Ewing sarcoma anatomic sites should be considered for surgery only?
- Expendable sites -- Proximal fibula -- Lateral 4/5th of the clavicle -- Parts of the ilium
62
Which syndromes are a/w Wilms tumor?
- 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
What are the standard borders of an abdominal RT field for Wilms tumor?
- AP/PA fields -- Sup: diaphragm -- Inf: Bottom of obturator foramen -- Block femoral heads
64
What is the general tx paradigm for a Wilms tumor?
- COG: -- Upfront resection -- CHT ± RT - SIOP: -- Neoadj. CHT -- Resection
65
Which histologies are unfav. in Wilms tumor?
- Unf: -- Anaplasia (dense nuclear chromatin, nuclear pleomorphism, mitotic figures) -- Clear cell sarcoma of the kidney -- Rhabdoid tumor of the kidney.
66
What % of pts present w/ fav histology Wilms tumor?
- Fav: 90% -- 2-yr mortality: 7.1% - Unfav: 10% -- 2-yr mortality: 44%
67
What is the EFS and OS benefit for Wilms tumor pt w/ lung nodules seen on CT but *not* CXR?
- 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
For a Wilms tumor, what is the % bx rate of lung nodules identified on a CT scan?
- Overall: ~75% - Isolated lung lesion: ~82% - Multiple lung lesions: ~69%
69
What is the brain RT doses for a Wilms tumor pt w/ brain metastases?
- 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
What % of RMS are embryonal?
60-70%
71
What % of RMS are alveolar?
20-30%
72
How does the prognosis of RMS relate to histology?
- Favorable prognosis -- Boytroid and Spindle Cell - Intermediate prognosis -- Embryonal - Worst prognosis -- Alveolar
73
What are the pathognomonic findings of Ewing sarcoma on plain film?
- Onion skinning -- aka Moth- eaten -- aka Permeative
74
What are the pathognomonic findings of Osteosarcoma sarcoma on plain film?
Sunburst pattern
75
What are the typical locations of Ewing sarcoma?
The diaphysis of long bones
76
What are the typical locations of Ewing sarcoma?
Metaphysis (near growth plates) of long bones
77
Which mutations are a/w embryonal RMS of the GU tract?
DICER1
78
Which mutations are a/w alveolar RMS?
PAX3-and PAX7-FOXO1 gene fusions from t(2;13) or t(1;13)
79
Which mutations are a/w Ewing's sarcoma?
EWSR1 gene rearrangements t(11:22)
80
When should LN dissection be performed in boys w/para testicular RMS?
- < 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
What dose of RT is appropriate for children w/ painful bony metastases?
- < 16 yrs old → 25.2 Gy to tumor + 1 cm - ≥ 16 yrs old → 30.6 Gy to the whole bone
82
What is an ASKIN tumor?
PNET or Ewing sarcoma of the chest wall
83
What is the 5-yr OS for patients w/ localized vs. metastatic osteosarcoma?
- Localized: 75% - Metastatic: 25%
84
How are RT volumes defined for Ewing sarcoma per modern protocols?
- 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
How does N-MYC mutation affect EFS in neuroblastoma pts?
- +N-MYC → 3-yr EFR 10% - -N-MYC → 3-yr EFR 93%
86
How is focal anaplasia defined for Wilms tumor?
Anaplasia is confined to multiple clearly defined loci within the primary tumor
87
When is a bone marrow biopsy (BMBx) required for RMS?
- For all diagnoses - Usually required of all non-CNS peds malignancies besides CCSK, and Wilms tumor
88
When is a brain MRI obtained for RMS?
RMS of Parameninges
89
When is an LP done for RMS?
For suspected CNS involvement
90
What are the 5-ry EFS for low, intermediate, and high-risk RMS?
- Low: 85-90% - Int: 73% - High: 32%
91
Which sites are considered parameningeal for RMS?
- MMNNOOPP -- Middle ear -- Mastoid region -- Nasal cavity -- Nasopharynx -- Pterygopalatine fOssa -- Infratemporal fOssa -- Paranasal sinus -- Parapharyngeal region
92
What are the 10-yr OS for favorable, and unfavorable histology Wilms tumors?
- 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
What % of newly dx Wilms tumor cases have a family hx of the disease?
1-2%
94
What % of Wilms tumors are resectable at dx?
- 90-95%, hence surgery w/o bx is the first plan of attack
95
What are poor prognostic factors in Wilms tumor pts?
- Focal/Diffuse anaplasia - Gain of 1**q**, loss of 1p, loss of 16q
96
What is the median age of dx of NB?
22 mos

ROQs (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions