Wilms Tumor Flashcards
WT
Other name for WT?
Nephroblastoma
WT
WT is a highly curable childhood neoplasm with a current cure rate of about >90%.
TRUE or FALSE?
True
WT
WT is the most common malignant renal tumor or childhood.
TRUE or FALSE?
True.
WT
At what age is the peak incidence of WT?
between 3-4 years
WT
What syndromes are associated with WT?
- WAGR syndrome
- Denys-Drash syndrome
- overgrowth syndromes like Beckwith-Wiedemann and Simpson-Golabi-Behmel syndromes
WT
What are the associated clinical pictures in WAGR syndrome?
WT
Aniridia
Genitourinary malformations
Retardation (mental)
WT
What are the associated clinical pictures in Denys-Drash syndrome?
pseudohermaphroditism
mesangial sclerosis,
renal failure
WT
WT
What are the associated clinical pictures in Beckwith-Wiedemann syndrome?
somatic gigantisim, omphalocele macroglossia, genitourinary abnormalities, ear creases, hypoglycemia, hemihypertrophy, predisposition to WT and other malignancies
WT
What chromosome is involved in WT1, a negative dominant ongogene related to the development of WT?
11p13
WT
What chromosome is involved in WT2, associated with Beckwith-Wiedemann syndrome and WT?
11p15.5
WT
What genetic abnormality is associated with higher relapse rates and mortality rates? (NWTS-5)
LOH 1p 16q
Patients with loss of heterozygosity (LOH) at 16q and 1p have higher relapse and mortality rates.
WT
The presence of these in children with very low-risk WT treated with just surgery alone, has been prospectively validated to be an important predictor of relapse.
WT1 mutation and 11p15 loss
WT
What is the most lethal renal neoplasm in children previously classified as WT?
RTK (rhabdoid tumor of the kidney)
WT
4-year survival of patients with stage IV WT with FA is double than that of stage II with DA.
TRUE or FALSE?
The 4-year survival rates for patients with stage II, III, and IV FA
were 90%, 100%, and 100%, compared with 55%, 45%, and 4%, respectively,
for patients with similar stage DA WT.
WT
What is the histologic feature that has greatest clinical significance in WT?
anaplasia
WT
What is the classic presentation of Wilm’s tumor?
painless abdominal swelling in a healthy child incidentally discovered by the child’s mother or by a physician on a routine physical examination.
WT
What are the first signs that WT spread beyond the pseudocapsule?
invasion into the renal sinus or the intrarenal blood and lymphatic vessels.
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Bilateral renal involvement
Stage V
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Thoracic lymph node involvement
Stage IV
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Abdominal lymph node involvement
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Pelvic lymph node involvement
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Previous open biopsy
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Previous biopsy (by FNA only)
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Spontaneous rupture prior to surgery
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Tumor spillage during surgery
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Rupture or spillage confined to the flank
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Bone metastases
Stage IV
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Liver metastases
Stage IV
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Brain metastases
Stage IV
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Lung metastases
Stage IV
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
peicemeal surgery
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Incidental tumor thrombi within the renal vein found after en bloc removal of tumor
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Peritoneal implants
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Tumor penetration through the peritoneal surface but no peritoneal surface implants
Stage III
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Completely resected, involvement of soft tissue of the renal sinus but no renal vessel involvement
Stage II
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Completely resected, involvement of soft tissue of the renal sinus and with renal vessel involvement
Stage II
WT
Identify the COG stage (assuming if other parameters are not mentioned, are not observed):
Completely resected, intact capsule
Stage I
WT
What study upstaged lymph node involvement from stage II to III?
NWTS-3
WT
What study downstaged tumor spill from stage III to II?
NWTS-3
WT
What study disregarded the hilar plane as the point of demarcation between stages I and II?
NWTS-5
WT
Identify the characteristics that can be treated with surgery alone according to COG risk group classification for favorable Wilms Tumor.
<2 y
<550 g
Stage I
Any LOH
WT
Identify the characteristics that can be treated with EE4A according to COG risk group classification for favorable Wilms Tumor.
Low risk group
Stages I and II “without” LOH
Stages I and II “with” LOH are treated with DD4A
Stages III and IV(rapid responder) “without” LOH are treated with DD4A
WT
Identify the characteristics that can be treated with regimen “M” (VAD/Cy,E,) according to COG risk group classification for favorable Wilms Tumor.
Higher risk group
Stages III and IV “with” LOH
Stage IV “without” LOH and slow response to DD4A
***
WT
What are the agents used in DD4A?
Vincristine, Actinomycin-D, Doxorubicin
VAD
WT
What are the agents used in EE4A?
Vincristine, Actinomycin-D
WT
What are the agents used in regimen M?
VAD Cyclophosphamide, Etoposide
WT
Proper staging of lymph nodes and contralateral kidneys must include lymph node sampling and exploration of the contralateral kidney.
TRUE or FALSE?
False.
Sampling of lymph nodes only.
Contralateral kidneys can be evaluated using imaging
WT
When do you administer WLI? (based on COG risk)
Stage IV FH WT with LOH that are slow responders
WT
For UH WT, (High Risk)
What is the COG recommended treatment for stage I,DA?
Nephrectomy+RT+DD4A
WT
For UH WT, (High Risk)
What is the COG recommended treatment for stage I,FA?
Nephrectomy+RT+DD4A
WT
For UH WT, (High Risk)
What is the COG recommended treatment for stages II-IV FA?
Nephrectomy+RT+DD4A
WT
For UH WT, (High Risk)
What is the COG recommended treatment for stages II-IV DA?
Nephrectomy+RT+UH1
+RT to metastatic sites
WT
For UH WT, (High Risk)
What is the COG recommended treatment for stages I-III CCSK?
Nephrectomy+RT+Regimen I
WT
For UH WT, (High Risk)
What is the COG recommended treatment for stages IV CCSK?
Nephrectomy+RT+UH1
+RT to metastatic sites
WT
For UH WT, (High Risk)
What is the COG recommended treatment for any stage CCSK?
Nephrectomy+RT+UH1
+RT to metastatic sites
WT
RT doses (as recommended by COG)
Stages I and II WT
None
WT
RT doses (as recommended by COG)
Stages III FH, stage I–III FA
10.8 Gy to the flank
WT
RT doses (as recommended by COG)
Stages I-II DA, stage I–III CCSK
10.8 Gy to the flank
WT
RT doses (as recommended by COG)
Stages III DA, stage I–III RTK
19.8 Gy to the flank
infants ≤12 months 10.8 Gy
WT
RT doses (as recommended by COG)
Recurrent abdominal WT
- 6–18 Gy (<12 months)b
- 6 Gy (older children, previous RT ≤ 10.8 Gy)
Boost dose of 9 Gy to gross residual tumor
WT
RT doses (as recommended by COG)
Lung metastases (FH)
WLI
12Gy/1.5/8
WT
RT doses (as recommended by COG)
Lung metastases (UH)
WLI
12Gy/1.5/8
WT
RT doses (as recommended by COG)
Brain metastases
- 6/1.8/17 (WBRT)
21. 6Gy (WBRT) + 10.8 IMRT or stereotactic boost (32.4 Gy total)
WT
RT doses (as recommended by COG)
Liver metastases
19.8 Gy / 1.8 /11
WT
RT doses (as recommended by COG)
Bone metastases
25.2 Gy + 3 cm margin
WT
RT doses (as recommended by COG)
unresected LN metastases
19.8 Gy (same total as liver, and flank RT to RTK, stage III DA)
WT
RT is not indicated for stages I and II favorable histology but is indicated for stages I and II with either focal or diffuse anaplasia.
TRUE or FALSE?
True.
WT
For UH WT, at what day should RT be started after surgery with surgery as day 0?
COG
Day 9 preferably but no later than day 14.
WT
What NWTS study eliminated the RT for stage II FH?
NWTS-3
WT
Is there a dose response for CCSK and anaplatic tumors?
Whether yes or no, site the NWTS studies.
NWTS-3 and NWTS-4 data showed no RT dose
response for CCSK and anaplastic tumors.
Therefore, it was decided to treat all
abdominal disease with 10 Gy.
except for stage III DA and stage I to III RTK, where a higher dose
of 19.8 Gy is recommended by the COG.
WT
When using parallel-opposed fields to the flank RT, why does the medial borders have to cross the midline to include the entire vertebra?
to minimize dose disturbances
WT
What is the PTV?
tumor bed (outline of the kidney and associated tumor on initial CT or MRI) + 1 cm margin.
WT
If the lungs need to be treated with the abdomen, it is preferable to include them in one treatment portal.
TRUE or FALSE?
True.
WT
Identify the trial
One of the major goals of the trial was to prospectively analyze the
prognostic significance of LOH at chromosomes 1p and 16q..
NWTS-5
1995-2001
WT
Identify the trial
Patients with stage I FH and anaplastic histology
and stage II FH tumors were treated with 18 weeks of dactinomycin and
vincristine without RT.
Stage I FH tumors in children younger than 24 months of
age and with tumor weight <550 g were treated with surgery alone.
Stage III and
IV FH and stage II to IV focal anaplastic tumors were treated with 24 weeks of
dactinomycin, vincristine, doxorubicin, and irradiation.
Stage II to IV diffuse
anaplastic tumors and stage I to IV CCSK were treated with cyclophosphamide,
vincristine, doxorubicin, and etoposide along with irradiation.
Stage I to IV RTK
was treated with carboplatin, etoposide, cyclophosphamide, and irradiation.
NWTS-5
1995-2001
WT
Identify the trial
The
RT guidelines were similar to those used in the previous NWTS study except for
anaplastic tumors, where a dose of 10.8 Gy to the flank and abdomen was recommended compared to an age-adjusted schedule used in the previous study.
NWTS-5
1995-2001
WT
Identify the trial
This was designed with cost containment in mind.
The results
proved that the survival was similar among patients who received standard
course (5 days) or single-dose, pulse-intensive (PI) dactinomycin chemotherapy.
Further, PI therapy was associated with less hematologic toxicity and marked
reduction of treatment costs
NWTS-4
1986-1994
WT
Identify the trial
The overall objective was to reduce therapy for low-risk patients
(stage I to III FH) and to intensify treatment by adding a fourth drug,
cyclophosphamide, for stage IV tumors with FH and all UH tumors.
NWTS-3
1979-1985
WT
Identify the trial
The results of this study demonstrated that RT and doxorubicin could be eliminated in children with stage II FH tumors.
Patients with stage III FH tumors who received doxorubicin or 20 Gy had fewer abdominal relapses than those receiving 10 Gy without doxorubicin.
The addition of cyclophosphamide in high-risk patients did not improve outcomes.
NWTS-3
1979-1985
WT
Identify the trial
This trial showed that in patients with group I tumors, there was no survival
difference between 6 months and 15 months of dactinomycin plus vincristine.
Patients with group II to IV tumors had a superior 2-year RFS of 77% with
doxorubicin, dactinomycin, and vincristine compared with 63% with
dactinomycin and vincristine alone.
NWTS-2
1974-1979
WT
Identify the trial
This trial showed that postoperative RT was not necessary for children younger
than 2 years of age with group I tumors and that combined dactinomycin and
vincristine for irradiated patients with group II and III tumors was better than
therapy with either agent alone. The RFS with and without RT among patients
with group I tumors younger than 2 years of age was 90% and 88%,
respectively.
NWTS-1
1969-1974
WT
Identify the study:
Patients with very-low risk WT treated with observation after nephrectomy have 4-year EFS of 90% and 4-year OS of 100%
AREN 0532
WT
Identify the study:
Patients with FH WT stages I to II with LOH had improvement in 4-year EFS from 75% to 84% with the addition of doxorubicin. (EE4A to DD4A)
AREN 0532
WT
Identify the study:
Patients with FH WT stages III with LOH had continued excellent outcomes with regimen DD4A.
AREN 0532
WT
RFS is worse for liver metastases compared to metastases to the lung and other sites.
TRUE or FALSE?
False
In a report from
NWTS-4 and NWTS-5, the RFS for patients with FH WT and liver metastases
was 76%, and this was similar to the RFS in patients with lung metastases
(76%), liver and lung metastases (70%), and metastases to other sites (64%).
WT
The performance of a tumor biopsy or the use of chemotherapy before definitive surgery is an indication for flank RT in BWT.
TRUE or FALSE?
False
WT
How much of each kidney must be preserved in order to perform the initial surgery in BWT?
> 2/3 of each
Initial surgical resections should be performed only if more than two-thirds of each kidney can be preserved.
WT
The tumor in each kidney is staged separately for bilateral WT.
TRUE or FALSE?
True.
WT
When do you do second-look and definitive surgery after upfront chemotherapy in BWT?
6 and 12 weeks
WT-CCSK
What are the independent prognostic factors for survival?
treatment with doxorubicin, tumor stage, age at diagnosis, and tumor necrosis
WT
What regimen is as effective as EE4A in preventing tumor rupture in the preoperative setting?
pre-op RT + dactinomycin
SIOP-5
WT
What are three common treatment-related late effects that contributed to mortalities in patients treated for WT?
secondary malignancy
congestive heart failure
end-stage renal disease
(from previous in-service exams)
- Congenital syndromes associated with Wilms, EXCEPT.
a. WAGR
b. Denys-Drash
c. Beckwith-Wiedemann
d. NF-1
D
(from previous in-service exams)
- Stage of Wilms if pre-op Chemo was or (+) tumor spillage before surgery
a. I
b. II
c. III
d. IV
e. V
C
(from previous in-service exams)
- In NWTS, adjuvant treatment strategy for Wilms Stage I/II Favorable Histology
a. chemo only
b. RT only
c. RT ff by chemo
d. Chemo ff by chemo
A
(from previous in-service exams)
- Recommended RT dose to the Whole Lung in Wilms tumor with Pulmonary Mets
a. 10.5 Gy
b. 12 Gy
c. 14 Gy
d. 6 Gy
B
(from previous in-service exams)
- Typical dose for Wilms Tumor who need local RT to the tumor bed and nephrectomy site and adjacent nodes
a. 10.5 Gy
b. 12 Gy
c. 14.4 Gy
d. 5.4 Gy
A
(from previous in-service exams)
- Other types of pediatric renal tumor with the worst prognosis based on NWTS IV
a. Clear Cell
b. Rhabdoid tumor of the Kidney
c. Renal Cell Carcinoma
d. Mesoblastic Nephroma
B
(from previous in-service exams)
- True or False: Based on NWTS, in Bilateral Wilms’, each kidney is staged separately.
True
(from previous in-service exams)
- Radiation Therapy for Wilms’ is ideally done within ____ week(s) post-op.
a. 1
b. 2
c. .3
d. 4
B
