tumors

Question 1

Answer 1

Question 2

Answer 2

Question 3

most common extrracranial solid tumor in childhood

Answer 3

neuroblastoma

Question 4

Esthesioneuroblastoma age distribution

Answer 4

bimodal: teens and middle age

Question 5

aggressive sun-burst periosteal rxn in the skull

Answer 5

metastatic neuroblastoma

Question 6

most common liver tumor of early childhood (and third most common overall)

Answer 6

hepatoblastoma

Question 7

hepatoblastoma multisystem disease/syndrome associations

Answer 7

• Beckwith-Wiedemann (q6 US screen is performed)
• FAP
• Fetal alcohol syndrome

Question 8

tumor marker elevated in hepatoblastoma

Answer 8

AFP

Question 9

pediatric tumors with calcs

Answer 9

• NEUROBLASTOMA (#1 most common overall peds tumor)
• hepatoblastoma ( #3 most common overall peds tumor, #1 MC in the liver)
• infantile hemangioma (hemangioendothelioma)

Question 10

most common tumors to metastasize to the kidneys in kids

Answer 10

• neuroblastoma
• leukemia
• lymphoma

Question 11

primative neural crest cell malignancy, sympathetic in origin

Answer 11

Neuroblastoma

Question 12

mets may be found in _______% of patients with neuroblastoma, and most commonly met to the _________

Answer 12

mets may be found in 75**% of patients with neuroblastoma, and most commonly met to the **bone

Question 13

Stages of Neuroblastoma

Answer 13

Question 14

Cortex or medullary cavity met better for Neuroblastoma prognosis?

Answer 14

medullary mets are better! aka, 4S stage is what you want

Question 15

what are the Neuroblastoma stages with the worst prognosis?

Answer 15

3 and 4

Question 16

What is neuroblastoma stage 4S?

Answer 16

age <1 year AND mets to:

• skin
• liver
• bone MARROW

Question 17

DWi characteristics of neuroblastoma

Answer 17

demonstrates diffusion restriction

(ganglioneuroblastoma does not!)

Question 18

wilm variant that mets to bones

Answer 18

Clear cell sarcoma

Question 19

Wilms variant with brain neoplasms

Answer 19

rhabdoid tumor

Question 20

“worst porognosis of all the renal tumors”

Answer 20

Rhabdoid tumor (wilms variant)

has brain neoplasms

Question 21

Beckwith-Wiedemann has increased risk of what kid tummy tumors?

Answer 21

• Wilms
• hepatoblastoma

You have to screen with abdominal US every 3 months until age 8

Question 22

Increased risk for Wilms and Hepatoblastoma is specifically associated with what syndrome?

Answer 22

Beckwith-Wiedman

Question 23

Horseshoe kidney increased risk for what malignancy?

Answer 23

2x increased risk of Wilm tumor

Question 24

most common childhood renal neoplasm?

Answer 24

Wilms tumor

Question 25

Wilms tumor age distribution

Answer 25

3-5

Question 26

Which patients should get regularly screened for Wilms?

Answer 26

* BW syndrome * WAGR * Horseshoe kidney (Turner's) * T18

Question 27

Stage of a kid with bilateral Wilms tumor at the time of diagnosis?

Answer 27

Stage 5 (this stage is unique to Wilms)

Question 28

Wilms tumor, aniridia, GU anomalies, mental retardation

Answer 28

WAGR syndrome

Question 29

what tumors arise from persistent metanephric blastema

Answer 29

Wilms and mesoblastic nephroma

Question 30

looks like a Wilms tumor but the kid is \< 1yo

Answer 30

mesoblastic nephroma

Question 31

Answer 31

Question 32

precursor to Wilms tumor

Answer 32

nephroblastomatosis frequent screening is performed

Question 33

NF1 associated childhood tumors:

Answer 33

* **Wilms** * **Rhabdomyosarcoma** * AML * leiomyosarcoma

Question 34

if you have a bilateral Wilm's tumor, what did you likely have prior?

Answer 34

nephroblastomatosis

Question 35

childhood tumor that extends into the renal pelvis

Answer 35

multilocular cystic nephroma "michael jackson tumor" uping kids and middle aged women

Question 36

differential for each age

Answer 36

Question 37

8 month old

Answer 37

Question 38

hepatoblastoma is associated with what overgrowth syndrome?

Answer 38

Beckwith Wiedemann

Question 39

classic clinical history of a kid with hepatoblastoma?

Answer 39

precocious puberty

Question 40

risk factor associated with development of hepatoblastoma?

Answer 40

prematurity

Question 41

infant. heart is structurally normal

Answer 41

hemangioendothelioma (infantile capillary hemangioma) with high flow HF treat with propanolol

Question 42

elevated vascular growth factor in an infant

Answer 42

hemangioendothelioma

Question 43

enlarging abdominal mass in a 1 year old

Answer 43

mesenchymal hamartoma Most children present in the neonatal period with an enlarging abdominal mass, with 80% diagnosed by 2 years of age Imaging: * large, * multicystic with **cysts of various sizes** * can contain debris, and * can be divided by septae of variable thickness. * **No calcifications** (well...rarely)

Question 44

enlarging liver mass in a kid \<1 without elevated markers

Answer 44

mesenchymal hamartoma

Question 45

cystic liver lesion in a kid \<1 without calcifications

Answer 45

mesenchymal hamartoma

Question 46

kid HCC occurs in pts with:

Answer 46

* **_alpha-1 anti-trypsin_** * **_glycogen storage diseases_** * tyrosinemia * **_biliary atresia_** * chornic viral hepatitis