Peds Gu Onc

Front 1

What is the staging for Neuroblastoma?

Back 1

International Neuroblastoma Staging System (INSS); based on clinical, radiographic, and surgical evaluation

Front 2

What is Stage I for neuroblastoma?

Back 2

I: localized tumor with complete gross excision, w or w/o micro residual dz; negative ipsilateral LN microscopically (nodes attached to and removed with the primary tumor may be positive)

Front 3

What is Stage II for neuroblastoma?

Back 3

IIA: localized tumor with incomplete gross excision; ipsilateral nonadherent LN negative microscopically
IIB: localized tumor with or w/o complete gross excision; positive ipsilateral nonadherent LN; enlarged contralateral LN must be negative

Front 4

What is Stage III for neuroblastoma?

Back 4

III: Unresectable unilateral tumor infiltrating across the midline, -/+ LN involvement; localized unilateral tumor with contralateral regional LN +; or midline tumor w bilateral extension by infiltration (unresectable) or by LN +

Front 5

What is Stage IV for neuroblastoma?

Back 5

IV: Any primary tumor with dissemination to distant LN, bone, bone marrow, liver, skin, and/or other organs
IV-S: localized primary tumor (as defined for stage I, IIA, IIB), with dissemination limited to skin, liver, and/or bone marrow (< 10% tumor) in infants < 1 yr of age

Front 6

What is neuroblastoma derived from?

Back 6

Most common extracranial solid tumor of childhood. Derived from neural crest cells that form the adrenal medulla and sympathetic chain.

Front 7

What chromosomal deletion is associated with neuroblastoma?

Back 7

deletion of short arm of chromosome 1 (70-80% of patients): it is a poor prognostic marker

Front 8

What are 2 poor prognostic indicators for neuroblastoma?

Back 8

1. deletion of short arm chromosome 1 (70-80% patients)
2. amplification of N-myc oncogent

Front 9

How often is metastasis present at dx in neuroblastoma?

Back 9

70%: most commonly in long bones and skull

Front 10

Paraneoplastic syndromes in neuroblastoma:

Back 10

1. catecholamine: mimic pheo
2. vasoactive intestinal polypeptide (VIP)
3. acute myoclonic encephalopathy

Front 11

Urinary metabolites of catecholamines in neuroblastoma:

Back 11

vanillylmandelic acid and homovanillic acid 90-95% of patients

Front 12

What are the prognostic factors in neuroblastoma?

Back 12

1. Age: infants < 1 better survival
2. Site: nonadrenal primaries better
3. Stage: overall survival stages 1, 2, or 4S 75-90%; 2yr survival for stage 4 = 19-30%
4. N-myc: rapid tumor progression and poor prognosis 30-40% with advanced dz (independent risk factor)

Front 13

What are the Tx for neuroblastoma?

Back 13

1. Surgery: >90% survival stage 1; defer until after chemotx for advanced dz
2. ChemoTx
3. XRT: no benefit for low stage; inc local control in advanced stage 4 or bulky stage 3

Front 14

What is the most common soft tissue sarcoma in infants and children?

Back 14

Rhabdomyosarcoma (RMS)

Front 15

Most common GU sites for RMS?

Back 15

15-20% of cases arise from GU system: prostate, bladder, paratesticular

Front 16

Age distribution for RMS?

Back 16

peak first 2 yrs and again at adolescence (bimodal)

Front 17

What is the Li-Fraumeni syndrome?

Back 17

-rare autosomal dominant d/o greatly inc susceptibility to cancer
-mutation of p53 tumor suppressor gene
-assoc b/w childhood sarcomas with moms who have premenopausal breast cancer and with siblings who have inc risk of cancer

Front 18

T/F: Neurofibromatosis is assoc with increased incidence of RMS.

Back 18

True

Front 19

Most common subtype of RMS?

Back 19

-embryonal RMS: arising from muscle grps (solid)
-sarcoma botryoides: polypoid variety develops in hollow organs or body cavities (i.e. bladder, vagina)
-spindle cell (leiomyomatous): paratesticular region
-botryoid and spindle cell: excellent survival
-LOH on chormo 11p15 --> overexpression of IGF-II --> tumor growth

Front 20

Alveolar RMS:

Back 20

-more commonly trunk and extremity
-worse prognosis: higher rate recurrence and mets
-chromo translocations: t(1;13), PAX7-FKHR younger, better prognosis
t(2;13), PAX3-FKHR adverse prognosis

Front 21

T/F: RMS extension to regional LN is uncommon.

Back 21

False

Front 22

Usual site of mets for RMS?

Back 22

lungs

Front 23

What is the "bladder sparing" approach to RMS Tx?

Back 23

Both chemoTx and XRT before surgical resection
-60% retained bladder function at 4 yrs from dx
-overall survival > 85%

Front 24

What is an exception to "bladder sparing" approach to RMS?

Back 24

tumors amenable to partial cystectomy at dx (dome or lateral bladder wall)

Front 25

Paratesticular RMS:

Back 25

-distal portion of spermatic cord
-unilateral painless scrotal swelling
-dx SUS

Front 26

T/F: RMS extension to regional LN is uncommon.

Back 26

False

Front 27

Usual site of mets for RMS?

Back 27

lungs

Front 28

What is the "bladder sparing" approach to RMS Tx?

Back 28

Both chemoTx and XRT before surgical resection
-60% retained bladder function at 4 yrs from dx
-overall survival > 85%

Front 29

What is an exception to "bladder sparing" approach to RMS?

Back 29

tumors amenable to partial cystectomy at dx (dome or lateral bladder wall)

Front 30

Paratesticular RMS:

Back 30

-distal portion of spermatic cord
-unilateral painless scrotal swelling
-dx SUS

Front 31

What stage is paratesticular RMS typically? What type of RMS?

Back 31

60-80 are stage I; >90% embryonal and have a good prognosis; alveolar histology has better prognosis vs alveolar at other sites

Front 32

How often is there RLN mets with paratesticular RMS?

Back 32

20%

Front 33

What surgical approach for paratesticular RMS?

Back 33

Radical inguinal orchiectomy

Front 34

When to do RPLND in paratesticular RMS?

Back 34

children > 10 yo for ipsilateral RPLND as part of staging before chemoTx (higher risk for RP relapse and worse overall survival)

Front 35

Why is RPLND in paratest RMS controversial?

Back 35

morbid surgery, false-pos rate 65-94%, chemoTx is effective in microscopic nodal dz

Front 36

What is survival rate for paratest RMS?

Back 36

multimodal Tx survival 90%; pts with clinically negative nodes treated with chemo

Front 37

Where does vaginal RMS usually occur?

Back 37

anterior vaginal wall at the embryonic vesicovaginal septum (urogenital sinus)

Front 38

Histologic type of vaginal RMS? Vulvar RMS?

Back 38

embryonal or botryoid: excellent prognosis
Vulvar: alveolar, but good prognosis bc tends to be localized

Front 39

Tx for vaginal/vulvar RMS?

Back 39

chemoTx +/- XRT --> repeat bx to assis response before delayed tumor resection (partial vaginectomy or vaginectomy with hysterectomy)

Front 40

Histology for uterine RMS?

Back 40

>90% embryonal

Front 41

Tx for uterine RMS?

Back 41

primary chemoTx and delayed local Tx

Front 42

What is the most common primary malignant renal tumor of childhood? What is it derived from?

Back 42

Wilms' tumor: embryonal tumor from remnants of immature kidney

Front 43

Incidence of Wilms' tumor?

Back 43

children <15yrs 7-10 per million

Front 44

Median age for Wilms' tumor?

Back 44

3.5 yrs; earlier age in males than females

Front 45

Syndromes associated with Wilms' tumor?

Back 45

1. Denys-Drash
2. WAGR
3. Horseshoe kidney (7fold risk)
4. Beckwith-Wiedemann
5. renal fusion anomalies, cryptorchidism, HPS

Front 46

What is Denys-Drash syndrome?

Back 46

male pseudohermaphroditism, renal mesangial sclerosis, nephroblastoma; most progress to ESRD; mutation 11p13 (WT1)

Front 47

What is Beckwith-Wiedemann syndrome (BWS)?

Back 47

excess growth at the cellular, organ (macroglossia, nephromegaly, hepatomegaly) or body segment (hemihypertrophy)

Front 48

Risk of Wilms' tumor with BWS and hemihypertrophy?

Back 48

4-10% with 21% bilateral

Front 49

T/F: Children with BWS and nephromegaly have the greatest risk for Wilms' tumor?

Back 49

True

Front 50

What is WAGR syndrome?

Back 50

Wilms' tumor, Aniridia, Genital anomalies, mental Retardation: most likely to have bilateral Wilms' tumor and at younger age; prone to renal failure if survive to puberty

Front 51

What is WT1?

Back 51

located on 11p13, encodes transcription factor for gene regulation during normal renal and gonadal development; loss of one copy = WAGR syndrome; dysfcn WT1 protein = Denys-Drash

Front 52

What is WT2?

Back 52

11p15, assoc with BWS

Front 53

Familial Wilms' tumor:

Back 53

1-2%; earlier age of onset, inc freq for bilateral dz;
FWT1: 17q
FWT2: 19q

Front 54

Other chromosomal abnormalities in Wilms' tumor?

Back 54

16q in 20%
1p 10%
Both with inc risk for relapse and death

Front 55

"Classic" Wilms' tumor histology:

Back 55

favorable histology: islands of compact, undiff blastema and the presence of variable epithelial diff in the form of embryonic tubules, rosettes, and glomeruloid structures separated by a significant stromal component (Triphasic)

Front 56

Anaplastic Wilms' tumor:

Back 56

nuclear enlargement, hyperchromasia of enlarged nuclei, abn mitotic figures
-rarely seen in kids <2yo
-assoc with resistance to chemoTx

Front 57

Nephrogenic rests:

Back 57

> 1/3 of resected Wilms' tumor has these precursor lesions

Front 58

Two categories of nephrogenic rests:

Back 58

1. Perilobar (PLNR): BWS (WT2, 11p15); higher risk of contralateral dz in kids < 12mos
2. Intralobar (ILNR): aniridia, DDS (WT1, 11p13)

Front 59

What is nephroblastomatosis?

Back 59

presence of multiple nephrogenic rests; diffuse overgrowth of PLNR can produce thick rind that enlarges the kidney, but preserves its original shape; prone to favorable histology Wilms' tumor and bilateral tumors

Front 60

Common locations for mets in Wilms' tumor (most to least common):

Back 60

pulmonary, hepatic, bone, rarely brain

Front 61

Is exploration of the contralateral kidney necessary in Wilms' tumor?

Back 61

No, if preoperative CT or MRI demonstrates normal kidney

Front 62

When is preoperative chemoTx recommended for Wilms' tumor?

Back 62

1. bilateral Wilms' tumor (initial bx then preop chemo)
2. inoperable tumors at surgical exploration
3. tumor extension into IVC above hepatic veins

Front 63

Poor prognostic chromosomal factors for Wilms' tumor?

Back 63

LOH for 16q (poorer 2 yr relapse free and overall survival rates);
LOH at either 1p or 16q with stage I or II have inc relative risk of relapse and death;
With stage III or IV tumors risk of relapse and death increased in LOH at both 1p and 16q

Front 64

Tx of bilateral Wilms' tumor:

Back 64

5% of children; initial bx followed by 6wks of chemoTx; repeat imaging then partial nephx during 2nd look; close long-term FU

Front 65

Tx if inoperable (Stage III) Wilms' tumor:

Back 65

pretreatment with chemo 6wks almost always makes tumor resectable

Front 66

What is the most common testis tumor in prepubertal children?

Back 66

Mature teratoma: benign clinical course; all 3 germ cell layers; testis sparing enucleation of mass

Front 67

What is the 2nd most common testis tumor in prepubertal children?

Back 67

yolk sac tumor; characteristic Schiller-Duval bodies; 90% pos AFP

Front 68

Leydig cell tumors:

Back 68

Reinke's crystals pathognomonic; precocious puberty; most common sex cord tumor; age 4-5; inguinal orchiectomy

Front 69

What is the most common tumor found in assoc with intersex d/o?

Back 69

gonadoblastoma: occurs in dysgenetic gonads and assoc with Y chromosome; 1/3 bilateral; 25% risk in mixed gonadal dysgenesis, incidence inc with age; germ cell degeneration into seminoma or nonseminomatous tumors; ALL streak gonads in gonadal dysgenesis should be removed

Front 70

What is the most common malignancies that spread to the testis in children?

Back 70

Leukemia (ALL) and lymphoma (follicular and Burkitt's)