Peds

Question 1

For Rhabdomyosarcoma, what is indicated RT dosing for groups I,IIA,IIB/C, and group III orbit based on ARST 0331 IRS-VI? when is RT administered?

Answer 1

RT indications<div>Group I: no RT</div><div>Group IIA: 36 Gy</div><div>Group IIB/C: 41.4 Gy</div><div>Group III orbit: 45 Gy</div><div><br></br></div><div>RT week 13 for all patients<br></br></div>

Question 2

what is current favored chemo for rhabdomyosarcoma?

Answer 2

on ARST 1431: VAC/VI = vincristine, actinomycinD, cyclophosphamide / vincristine, irinotecan

Question 3

rhabdomyosarcoma over 5cm should be boosted to what dose?

Answer 3

59.4 Gy (due to higher recurrence rate on D9803)

Question 4

what is benefit of delayed primary excision for rhabdomyosarcoma?

Answer 4

allows for lower RT dose (more tumor shrinkage), thus potentially less adverse effects in future<div><br></br></div><div>no local control benefit (tested on D9803)</div>

Question 5

what are Oberlin risk factors for rhabdomyosarcoma, investigated in ARST 0431 IRS-VI?

Answer 5

Oberlin risk factors:<div>-Age >10 years or <1 year</div><div>-unfavorable primary site of disease</div><div>-≥3 metastatic sites</div><div>-bone or bone marrow involvement<br></br></div>

Question 6

consider RT with vincristine <b>+irinotecan</b> for what patients with metastatic rhabdomyosarcoma?

Answer 6

consider for 0-1 Oberlin risk factors present, not if 2+ Oberlin risk factors<div><br></br></div><div>improved EFS compared to historical cohort on ARST 0431 IRS-VI, but unsure if due to RT with additional radiosensitizer or interval compression of chemo</div>

Question 7

is there a benefit to hyperfractionated RT in rhabdomyosarcoma?

Answer 7

no - tested on IRS-IV

Question 8

what was pt population on ADOD0031?

Answer 8

1712 pediatric pts w Hodgkin’s lymphoma, intermediate risk<div><br></br></div><div>[=anything other than Stage IA-IIA nonbulky which is low risk, and Stage IIIB, IVB which is high risk]</div><div><br></br></div><div>Stage IA-IIA with bulky disease or stage IB, IAE, IIB, IIAE, IIIA, IVA with or without bulky disease.</div><div><br></br></div><div>Bulky: ≥6cm nodal aggregate or >1/3 ratio mediastinal width</div>

Question 9

what was tested regimen in AHOD0031?

Answer 9

“ABVE-PC x2 (doxorubicin, bleomycin, vincristine, etoposide, cyclophosphamide, prednisone) →<div><br></br></div><div>CT at cycle 2 to assess for rapid early response (RER) of CR or PR, or slow early response (SER).</div><div><br></br></div><div> <span>•RERs:</span> ABVE-PC +2 then CT or PET eval</div><div><span><br></br></span></div><div><span>•CR</span>:</div><div>→21 Gy/ 15 fx IFRT</div><div>vs.</div><div> <span>→obs</span></div><div><span><br></br></span></div><div><span>•less than CR</span>:</div><div>IFRT in all</div><div><br></br></div><div> <span>•SERs:</span></div><div><span></span>→DECAx2 + ABVE-PCx2 + 24 Gy IFRT </div><div>vs.</div><div>→ABVE-PCx2 + 21 Gy IFRT</div>”

Question 10

where is first relapse in pediatric Hodgkin’s lymphoma amongst patients with any response to first line chemo?

Answer 10

First relapse is usually at initial nodal sites, bulky or nonbulky, and rarely out of field<br></br><div>(from AHOD0031)</div>

Question 11

Based on AHOD0031, RT can be eliminated for what pediatric Hodgkin’s lymphoma pts?

Answer 11

“<span>•RERs with CR</span><span>:</span><div><span>No benefit in EFS with IFRT</span></div><div><span>4-yr EFS 88% IFRT vs. 84%, p=.11</span></div><div>-For PET defined CRs, EFS 87% both arms</div><div>-OS 99% in both arms (primary endpoint is EFS)</div>”

Question 12

what patients - even with RER - still should have IFRT according to AHOD0031?

Answer 12

“<span>Those with both anemia and bulky Stage I/II disease benefited from RT</span><div>4-yr EFS 89% vs. 78%</div>”

Question 13

what was IFRT dosing in AHOD0031?

Answer 13

21 Gy / 1.5 fx, AP/PA

Question 14

how was rapid early response assessed in AHOD0031?

Answer 14

RER was sum of perpendicular diameters of <60% of all volumes after cycle 2

Question 15

how was complete response defined in AHOD0031?

Answer 15

≥80% improvement in sum of perpendicular diamteters, or a return to normal size, plus no residual MS mass >2.0 cm, and negative gallium or PET.

Question 16

what stages are low risk pediatric Hodgkin’s lymphoma?

Answer 16

I, IIA

Question 17

pt population of CCG5942 (ped hodgkin’s lymphoma)

Answer 17

826 pts with low risk HL - stage I or IIA

Question 18

what was tested regimen in CCG5942?

Answer 18

COPP/ABV → if CR →<div>→21 Gy IFRT</div><div>vs.</div><div>→no IFRT</div>

Question 19

if IFRT necessary for low risk pediatric hodgkin’s lymphoma with complete response after COPP/ABV?

Answer 19

no. IFRT improves PFS but not OS.<div><br></br></div><div>10-yr EFS 91% obs vs. 83% IFRT 21 Gy, p=.004</div><div><b>10-yr OS ~97%, not different</b></div><div><br></br></div><div>risk factors for worse EFS were bulky, B symptoms, or nodular sclerosing<br></br></div>

Question 20

current standard chemo for Ewings sarcoma

Answer 20

VDC-IE q2weeks<div><br></br></div><div>vincristine, doxorubicin, cyclophosphamide - ifosphamide, etoposite</div>

Question 21

for Ewing’s sarcoma, local control obtained with surgery vs RT is similar vs different with what sites?

Answer 21

5-yr LF 4% surgery<div>15% RT (p<0.01)</div><div>7% S+RT (p=0.12)</div><div><br></br></div><div>Worse LF with use of RT for pelvis and extremity, and age ≥18.</div><div>Other sites (axial non-spine, spine, extraskeletal tumors) have similar LF with S vs. RT</div><div><br></br></div><div>Tumor size did not correlate with LF</div><div><br></br></div><div>from COG meta-analysis of 956 patients</div>

Question 22

if pt with lung metastases from Wilm’s with favorable histology (and no LOH) has complete response of pulmonary nodules to chemo, is Whole lung irradiation necessary?

Answer 22

4-yr EFS 80% [vs. 85% in historical control]<div>4-yr OS 96% Expected vs. observed events</div><div>15% vs. 20%, p=0.052</div><div><br></br></div><div>from COG AREN0553</div>

Question 23

what was pt population in COG ACNS0331?

Answer 23

549 pts w standard risk medulloblastoma

Question 24

what was tested regimen in COG ACNS0331 for medulloblastoma?

Answer 24

→23.4 Gy CSI plus 30.6 Gy posterior fossa boost to 54 Gy total with cis/CPM <div>vs.</div><div>→same but with 18 Gy CSI in ages 3-7 and IFRT in ages 2-21</div>

Question 25

can CSI be dose de-escalated to 18 Gy from 23.4 Gy for standard risk medulloblastoma? why not?

Answer 25

“no, based on ACNS0331.<div><br></br></div><div><span>Low dose CSI worse than standard dose</span> For SD-CSI vs. LD-CSI, <span>5-yr EFS 82.6%</span> vs. 72.1%<br></br></div><div><br></br></div><div>Isolated DF 12.8% for LDCSI and 8.2% for SDCSI.<br></br></div>”

Question 26

can IFRT replace boosting entire posterior fossa for medulloblastoma?

Answer 26

yes, from ACNS0331<div><br></br></div><div>For PFRT vs IFRT,</div><div>5-yr EFS 80.8% vs. 82.2%</div><div><br></br></div><div>5-yr OS 85.2% vs. 84.1%<br></br></div>

Question 27

if pt with grade 2-3 ependymoma has STR, can RT be delayed by administering chemo first?

Answer 27

no<div>5yr EFS 37%, vs 67% for those who had near GTR and underwent immediate RT</div><div><br></br></div><div>from ACNS 0121</div>

Question 28

is biopsy required to diagnose germinomas?

Answer 28

no, can be diagnosed by labs and imaging alone

Question 29

what is standard regimen for treating NGGCT?

Answer 29

from ACNS0112<div><br></br></div><div>cis/etoposide alt cis/ifosphamide x6 → CSI 36 Gy plus boost to 54 Gy</div><div><br></br></div><div>45 Gy boost to mets</div>

Question 30

can CSI be converted to whole ventricle RT if pt with NGGCT has complete response to chemo?

Answer 30

no<div>trial ACNS1123 (with no CSI if CR to chemo) closed early due to increased rate of failures, all in spine</div>

Question 31

“what are 3 noninferior treatment regimens for DIPG as tested in Cairo? what is one caveat to ““noninferior”” status?”

Answer 31

39 Gy / 13 fx<div>45 Gy / 15 fx</div><div>54 Gy / 30 fx</div><div><br></br></div><div>39 Gy / 13 fx had worse outcomes in pts <5yo</div>

Question 32

what are 2 treatment regimens for localized germinoma? is PFS/OS different?

Answer 32

CSI 24 Gy + boost 16 Gy<div>vs.</div><div>induction carbo/etoposide alt etoposide/ifos then 40 Gy IFRT<div><br></br></div><div>showed better 5yr PFS (97 vs 88%) with CSI</div><div><br></br></div><div>OS equivalent 90-95%</div></div>