Section 2: Pediatric Cancers - Tumors Of The Kidney

Front 1

What type of tumors are most kidney tumors?

Back 1

Wilm's tumor

Front 2

What are Wilm's tumors?

Back 2

tumors of kidney that are large, rapidly growing, vascular abdominal tumors

Front 3

what are Wilm's tumors frequently encapsulated by?

Back 3

Gelatinous capsule

Front 4

What 2 categories divide Wilm's tumors?

Back 4

1. Favorable Histology
2. Anaplastic

Front 5

What constitutes a histologic favorable Wilm's tumor?

Back 5

1. Undifferentiated, primitive development
2. Tissue is stromal, epithelial, & blastemic (precursor to stromal & epithelial cells)

Front 6

What cellular changes does an anaplastic Wilm's tumor have?

Back 6

Anaplastic nuclear changes

Front 7

What percentage of Wilm's tumor are 'anaplastic'?

Back 7

5%

Front 8

T or F: It is more common in younger children

Back 8

FALSE: more common in older children

Front 9

At what age does an anaplastic Wilm's tumor increase in frequency?

Back 9

- >5yrs of age, frequency increases
- Rare in first 2yrs of life

Front 10

What percentage of patients >5yrs of age have an anaplastic Wilm's tumor?

Back 10

13% of all Wilm's tumors in children who are 5yrs old and older

Front 11

T or F: Wilm's tumor, anaplastic, is significantly more frequent in Caucasian's than any other ethnicity

Back 11

FALSE: Significantly higher in African American children (vs caucasian)

Front 12

Does focal anaplasia indicate poor prognosis in an anaplastic Wilm's tumor?

Back 12

No, diffuse anaplasia indicates poor prognosis

Front 13

Why does diffuse anaplasia mean a poor prognosis?

Back 13

Anaplasia is assoc. w/resistance to chemo

Front 14

What is the second most common pediatric renal neoplasm?

Back 14

Clear-cell sarcoma of the kidney

Front 15

Is it better or worse than a favorable histologic Wilm's tumor?

Back 15

clear-cell has
1. higher rate of relapse
2. higher rate of death

Front 16

Does clear-cell sarcoma of the kidney every metastasize?

Back 16

Yes - associated w/wider distribution of metastases than Wilm's
- brain, bone, soft tissue
- extended presentation of metastases too

Front 17

What tumor of the kidney is rare, highly malignant, and may metastasize to brain & lungs?

Back 17

Rabdoid tumor of the kidney

Front 18

What patient population does Rabdoid tumor occur most frequently?

Back 18

Infants
85% occur before 2yrs of age

Front 19

Why do more than 80% of children die within the first year after Rhabdoid tumor of the kidney has been diagnosed?

Back 19

typically, wide metastases at presentation

Front 20

What are nephrogenic rests?

Back 20

precursor cells to Wilm's tumor

Front 21

What are nephroblastomatosis? and how are they classified?

Back 21

Nephroblastomatosis = multiple nephrogenic rests
- they are classified by position in kidney [ILNR= intralobar; PLNR = perilobar]

Front 22

Is renal cell carcinoma common in children?

Back 22

No - it's a rare subtype seen in teens & young adults
> in MALEs

Front 23

When are children at risk for renal cell carcinoma?

Back 23

If have genetic predisposition to it

Front 24

What tumor of the kidney is highly lethal & practically only seen in patients w/sickle-cell, more commonly in sickle trait?

Back 24

Renal medullary carcinoma
[median age 13yrs]

Front 25

What tumor of the kidney is a distinctive neoplasm in infancy?

Back 25

Congenital Mesoblastic Nephroma
[median age 2 months]

Front 26

T or F: Congenital Mesoblastic Nephroma occurs in twice as many males as females?

Back 26

True
[5% of childhood kidney tumors]

Front 27

Why is surgical resection an important treatment of Congenital mesoblastic nephroma?

Back 27

has a POOR response to chemo & XRT

Front 28

What is the incidence of Wilm's tumor?

Back 28

- 6% of childhood cancers
- slightly less freq. in boys
- 2-3yrs of age peak diagnosis
- rare in kids >5yrs

Front 29

What is the mean age of bilateral Wilm's tumor diagnosed?

Back 29

boys = 29.5 months
girls = 32.6 months

Front 30

What congenital malformations are associated with tumors of kidneys?

Back 30

1. Aniridia (congenital absence of iris)
2. Hemihypertrophy
3. Genital or renal malformations
4. Beckwish-Wiedemann syndrome (macroglossia, omphalocele, hemihypertrophy, visceromegaly)
5. Over growth syndromes

Front 31

What chromosomal abnormality is seen frequently in Wilm's tumor patients & always in pts w/aniridia?

Back 31

Gene deletion 11p13

Front 32

What is one hypothesis of the etiology of a Wilm's tumor?

Back 32

Wilm's tumor suppressor gene (WT1), located at 11p13, suppresses activity of tumor-transforming gene located elsewhere on chromosome 11.

Front 33

What gene deletion is seen in Beckwith-Wiedemann syndrome & found at 11p15?

Back 33

second Wilm's tumor gene (WT2)
alterations on 11p15 may predispose pt. to Wilm's tumor

Front 34

What is the percentage of p53 incidence in Wilm's tumors?

Back 34

75% have p53 mutation

Front 35

What do 20-30% patients present with in kidney tumor?

Back 35

Pain, malaise, hematuria

Front 36

What do 25% of patients present with a kidney tumor?

Back 36

Hypertension (d/t incr'd renin activity)

Front 37

How do patients present when a sudden subcapsular hemorrhage has occurred?

Back 37

Rapid abdominal distention
Anemia
Hypertension
Eggshell-type calcifications on x-ray

Front 38

What are the most common sites of metastases in tumors of the kidney?

Back 38

Lungs
Liver
Lymph nodes (regional)

Front 39

What percentage of patients diagnosed have involvement of both kidney's?

Back 39

7%

Front 40

What inidcates brain metastases?

Back 40

Neurologic signs

Front 41

T or F: a primary tumor of the kidney can extend to the inferior vena cava or right atrium

Back 41

TRUE

Front 42

What can bone pain at time of diagnoses indicate in tumors of the kidney?

Back 42

Bone metastases (often in long bones)

Front 43

What is hemihypertrophy?

Back 43

Where one side of body stops growing (includes organs)

Front 44

Other than assessing tumor size, why else is an abdominal US indicated at time of diagnosis?

Back 44

check for presence of thrombus in inferior vena cava & right atrium

Front 45

What kidney tumor diagnoses indicate a need for brain imaging?

Back 45

1. Clear-cell sarcoma
2. Renal-cell carcinoma
3. Rhabdoid of kidney

Front 46

What is NOT included on routine tumor assessment when Wilm's has been diagnosed?

Back 46

Assessment for liver, bone, and brain metastases
- only warranted by presenting s/s

Front 47

What is the most significant prognostic consideration of Wilm's tumors?

Back 47

Favorable vs anaplastic

Front 48

In addition to metastatic disease, what other involvment indicates poor prognosis?

Back 48

Lymph node involvement

Front 49

What cohort of patients have a significantly better prognosis than older children?

Back 49

- Entered in National Wilms' Tumor Study (NWTS)-1
- younger than 24months

Front 50

Read Table 2-12 on page 45

Back 50

Causes of Wilms' Tumor

Front 51

What patients with a favorable histology tumor have an increased risk of relapse & death?

Back 51

Tumor-specific loss of heterozygosity (LOH) for both chromosomes 16q and 1p in NWTS-5

Front 52

How are they using the identification of LOH in those with favorable histology to determine treatment?

Back 52

LOH for these chromosomal regions together with disease stage helps MD to weigh intensity of treatment against risk of treatment failure.

Front 53

In what year did the National Wilms Tumor Study begin?

Back 53

1969

Front 54

What was the overall relative 5-year survival rate for children with Wilms' tumor between 1985 and 1994?

Back 54

92% (improved from 81% btwn 1975 and 1984)

Front 55

What is used at the typical initial treatment for kidney tumors?

Back 55

Transperitoneal approach and nephrectmy

Front 56

What must also be assessed upon initial tumor assessment?

Back 56

Contralateral kidney
Sample lymph nodes

Front 57

Is small amounts of residual tumor associated with decreased survival rate?

Back 57

No

Front 58

What patients are considered for preoperative chemotherapy?

Back 58

1. Intravascular spread of disease
2. Very large invasive tumors
3. Pt. w/anesthesia-related risk

Front 59

What does preoperative chemotherapy prevent?

Back 59

Adequate staging assessment

Front 60

What stage tumor is given to patients whose tumors are ONLY staged on basis of imaging?

Back 60

Stage III tumor

Front 61

What is the common treatment approach when bilateral disease is present?

Back 61

1. Biopsy & initial staging of both kidneys
2. 6 weeks of chemo
3. nephrectomy of more invovled side
4. partial nephrectomy of other kidney

Front 62

What percentage of patients with bilateral disease will have residual tumor in remaining kidney after surgery?

Back 62

75%

Front 63

If there is residual tumor in remaining kidney are chances of survival lower?

Back 63

No

Front 64

Read Tabe 2-13, page 46

Back 64

National Wilms' Tumor Study (NWTS) Group Staging for Renal Tumors

Front 65

Chemotherapy options are based on NWTS- V & current studies

Back 65

see page 46
AREN0532 (very low, low, standard risk FH Wilm's)
AREN0533 (higher-risk FH Wilm's)
AREN0321 (high-risk renal)

Front 66

There are different chemo regimens for stages w/specific diagnosis - following shows range of different chemo regimens by stage & diagnosis

Back 66

Stage I, favorable or anaplastic
Stage II, favorable
Stage III,favorable
Stage IV, favorable
Stage V, individualized

Front 67

Why is the port extended to cross the midline?

Back 67

To prevent scoliosis

Front 68

What FH (Favorable histology) tumors require radiation therapy?

Back 68

Stage III and IV disease

Front 69

What change may be needed after lung irradiation?

Back 69

Decrease doses of chemotherapy

Front 70

When is whole abdomen radiation used?

Back 70

pts who had
1. tumor spillage during surgery
2. diffuse peritoneal seeding

Front 71

What percentage of patient's relapse with FH Wilms' tumor and anaplastic?

Back 71

15% w/FH Wilms' tumor
50% w/anaplastic Wilms' tumor

Front 72

In what time frame is there less chance of a cure if a recurrence appears?

Back 72

within 12months of diagnosis

Front 73

What patients have very poor prognosis if relapse or recurrence occurs?

Back 73

1. abdominal recurrence after XRT
2. Doxorubicin (Adriamycin) tx
3. Stage II - IV anaplastic histology at diagnosis

Front 74

What are the most common sites of disease recurrence with all stages?

Back 74

1. Lungs & pleura
2. Tumor bed
3. Liver

Front 75

Where do less frequent recurrences occur?

Back 75

1. Bone
2. Brain
3. Distant lymph node

Front 76

When do approximately 90% of recurrence occur?

Back 76

within 1st 2 years after diagnosis, remainder occur in next 2 years

Front 77

Why should children in a more favorable group be treated aggressively with relapse?

Back 77

Generally they have good response to retrieval therapy

Front 78

Why is longer follow-up indicated for clear-cell carcinoma patients?

Back 78

Relapses have been known to occur as long as 5 years after diagnosis