27: Childhood diseases III

Question 1

benign fibrous tumor in which the cells express muscle specific actin

Answer 1

infantile myofibromatosis

Question 2

most common fibrous tumor in infants

Answer 2

infantile myofibromatosis

Question 3

myofibroblast cells infiltrate skeletal muscel- beinign tumor consisting of fibroblasts and myofibroblasts- does not metastasize but can grow very large and infiltrate

Answer 3

aggressive infantile fibromatosis

Question 4

grade III fibrosarcoma has high grade atypia and high mitotic index- very hyperchromatic

Answer 4

fibrosarcoma

Question 5

most teratomas are…

Answer 5

sacrococcygeal
girls
benign

association with congenital malformations in 10% of sacrococcygeal teratomas

Question 6

most common solid tumor in the newborn

Answer 6

sacrococcygeal teratoma

Question 7

the most common solid congenital malignancy

Answer 7

congenital neuroblastoma

Question 8

neoplasm with all three germ layers

Answer 8

teratoma

Question 9

malignancy occurs in ____% of teratomas with immature tissue microscopically

Answer 9

12%

Question 10

malignancy is the ______ leading cause of death from disease in age 5-14

Answer 10

second

Question 11

childhood malignancy common origins

Answer 11

hamtopoietic
nervous
adrenal
renal

Question 12

10% of all childhood cancers

Answer 12

neuroblastoma

malignant tumor of primitive sympathetic cells

adrenal medulla and sympathetic ganglia

Question 13

abdominal mass and weight loss

Answer 13

neuroblastoma

Question 14

increased catecholamines in blood and urine

Answer 14

neuroblastoma

Question 15

metabolites VMA and homovanilic acid HVA in urine

Answer 15

neuroblastoma

Question 16

blueberry muffin baby

Answer 16

neuroblastoma

greatly distended abdomen with numerous bluish subcutaneous nodules of tumor

Question 17

• small blue round cells
• rosette structure
• dense core neurosecretory granules

Answer 17

neuroblastoma pathology

Question 18

dense core neurosecretory granules EM

Answer 18

neuroblastoma

Question 19

most common primary malignant tumor of the kidney in children

Answer 19

wilms tumor

Question 20

• abdominal mass
• hematuria
• fever
• hypertension

Answer 20

wilms tumor

Question 21

WAGR syndrome

Answer 21

33% risk for Wilms tumor

associated with a germline deletion and aniridia(no iris) and retardation

Question 22

Deny-Drash syndrome

Answer 22

90% risk to have WT

• WT mutation
• neuropathy, gonad problems

Question 23

most common sarcoma of childhood

Answer 23

rhadomyosarcoma

accounts for 5-8% of childhood malignancy

Question 24

‘best’ type of rhabdomyosarcoma

Answer 24

sarcoma botryoides/embryonal rhabdomyosarcoma

Question 25

‘worst’ type of rhabdomyosarcoma

Answer 25

pleomorphic rhabdomyosarcoma

overall, 50% of the children diagnosed with rhabdomyosarcoma survive 5 years

Question 26

tumor is traversed by a network of fibrous septae that divide the cells ito clusters or aggregates, creating a crude resemblance to pulmonary alveoli

Answer 26

alveolar rhabdomyosarcoma

Question 27

• growth retardation
• microcephaly
• short palpebral fissures
• maxillary hypoplasia
• atrial septal defect

Answer 27

FAS fetal alchohol syndrome

Question 28

• epicanthal fold
• underdeveloped jaw
• small eye openings
• smooth philtrum
• flat midface

Answer 28

FAS

Question 29

prevent neural tube defects

Answer 29

folic acid

prenatal screening test for neural NTDs- maternal serum AFP

Question 30

indicator of fetal lung maturity

Answer 30

surfactant