Ped Tumors

Question 1

Most common malignancy in childhood

Answer 1

ALL –> most are also B cell

Question 2

adolescent males, mediastinal mass

Answer 2

T cell leukemia (lymphoblastic)

Question 3

Trisomy 21

Answer 3

higher incidence of M4 AML

Question 4

Presents in jaw in Africa

Answer 4

Burkitts lymphoma –> B cell in origin
t(8;14)
(present in the abdomen in the US)

Question 5

Langerhans Cell Histiocytosis

Answer 5

Eosinophilic Granuloma
Letterer-Siwe disease
contain Birbeck granules (tennis racket handles)

Question 6

Ped brain tumors

Answer 6

below the tentorium

Question 7

Medulloblastoma

Answer 7

occur in the cerebellum

neuroendocrine tumor

Question 8

Astrocytoma

Answer 8

benign in children

Question 9

Ependymoma

Answer 9

tumor of cells lining ventricles

Question 10

Craniopharyngiomas

Answer 10

dx/dy from Rathke’s pouch near pituitary

difficult to remove

Question 11

most common solid tumors in children

Answer 11

brain tumors

Question 12

Pinealoblastoma

Answer 12

pineal gland tumor
poor prig
RARE in children

Question 13

Metastatic lesions

Answer 13

rare in the brain in children

usually neoplasms are primary

Question 14

Two tumors with abdominal masses in children

Answer 14

Neuroblastoma

Wilms Tumor

Question 15

second most common solid tumor in children

Answer 15

Neuroblastoma

Question 16

Neuroblastoma

Answer 16

adrenal medulla
also seen in sympathetic gangl –> paraganglioma –> posterior mediastinum
secrete HVA (catecholamine)
can mature to benign tumor
small round blue cell tumor –> forms rosettes

Question 17

Neuroblastoma cont’

Answer 17

prognosis worse in older children (4-5yrs)
can be congenital –> two-hit hypothesis
deletion short arm of chromosome 1
amplification of N-myc gene

Question 18

most common intra-abdominal tumor in children

Answer 18

Wilms’ Tumor

11p13 deletion/mutation

Question 19

Wilms’ Tumor

Answer 19

tries to make a kidney
primitive looking tissue
nodular renal blastema also seen
THE YOUNGER THE BETTER
two-hit hypothesis

Question 20

Denys-Drash Syndrome

Answer 20

kidney disease at birth
Wilms’ tumor
malformation of the sexual organs

Question 21

Beckwith Wiedemann Syndrome

Answer 21

hemihypertrophy
omphalocele
large tongue
–equivalent of first hit

Question 22

WAGR complex

Answer 22

deletion of gene on p arm of chromosome 11
Wilms' tumor
aniridia
genitourinary anomalies
mental retardation syndrome

Question 23

Most common soft tissue neoplasm in children

Answer 23

rhabdomyosarcoma

Question 24

Rhabdomyosarcoma

Answer 24

eosinophilic cyto
striations in cyto
muscle specific actin +
desmin +

Question 25

Alveolar rhabdomyosarcoma

Answer 25

t(2;13)
Sarcoma Botryoides –> embryonal form
protrudes from vagina

Question 26

Hemangioma

Answer 26

benign tumors of blood vessels
can spontaneously regress
malignant : angiosarcoma

Question 27

Osteogenic sarcoma

Answer 27

most common in adolescents

distal femur and prox tibia

Question 28

Ewing’s Sarcoma

Answer 28

small round blue cell tumor

t(11;22) –> MIC2

Question 29

Teratoma

Answer 29

ovaries 
mediastinum
sacro-coccygeal tertoma
benign is CYSTIC form
immature teratomas of ovaries usually seen in children

Question 30

most common neoplasm in infant testes

Answer 30

endodermal sinus tumor (yolk sac tumor)

secretes AFP

Question 31

Embryonal Ca

Answer 31

germ cell neoplasm in children

Question 32

Choriocarcinoma

Answer 32

germ cell neoplasm in children

secretes b-HCG

Question 33

Retinoblastoma

Answer 33

two-hit hypothesis
sporadic; not hereditary
first hit –> deletion of RB1 gene on chromosome 13
see leukocoria