2. pediatri abdominal masses

Question 1

Most common cancer in infants nedir?

Answer 1

Neuroblastoma

Question 2

Neuroblastoma’nın özellikleri nelerdir?

Answer 2

Most common cancer in infants

Genetic links/factors involved
N-myc
oncogene, chromosome deletion

Question 3

whaty is Most common cancer in infants

Answer 3

Neuroblastoma

Average age is 18 months; 80% < 5

Question 4

Clinical Presentation of neuroblastoma?

Answer 4

Pain, abd mass, other masses, malaise; skin

Can occur anywhere in sympathetic NS

Metastatic to lymph nodes, bone, BM, liver

Fever and malaise
catecholamine secretion

HTN, sweats, irritability; diarrhea;

opsoclonus-myoclonus; cerebellar ataxia

Question 5

How to diagose neuroblastoma?

Answer 5

catecholamine related:
htn, flushing, sweating, irritability); weight loss, pain, limp

preorbital ecchymosis, cutaneous nodules; abd mass; weakness/paralysis

CT/MRI to locate tumor; bone scan;MIBG;
PET—>for metastasis

Labs (urinary catecholamines);
homovanillic acid (HVA) and vanillylmandehc acid (VMA)

The International Neuroblastoma Risk Group (INRG) Staging System (INSS)used for stage

rakun göz

Question 6

Neuroblastoma Staging?

Answer 6

1. Localized tumor; complete excision

2 A Unilateral, incomplete gross resection; negative microscopic nodes

2B Unilateral, positive ipsilateral nodes; negative contralateral

3 Across midline, or contralateral nodes

4 Dissemination: bone marrow, liver, skin, bones

4S <1y: local stage 1-2 with mets to BM, liver, skin

Question 7

Treatment and Prognosis of neuroblastoma

Answer 7

Surgery: debulk or total removal; curative in low-stage disease; 2nd-look after other Rx

Chemotherapy – often platinum basedmulti-agent ~ stage

RT: to primary tumor site; NB cells very radiosensitive;

Question 8

what is High-risk neuroblastoma

Answer 8

long term survival rates between 25% and 35%

Highdose chemotherapy with autologous stem cel cellrescue–Surgery–Radiation

13-c / s retinoic acid ( isotretinoin , Accutane

Question 9

böbrek tümörleri nelerdir?

Answer 9

primarly Wilms, rapidly growing vascular abdominal tumors

clear cell sarcoma
renal cell CA, lymphoma, PNET, rhabdoid

Question 10

Incidence and Etiology of kidney tumors

Answer 10

5-6% of peds cancer

Higher in AA, lower in Asians

Peak age at 2-3;

1.5% familial in origin; associated with aniridia, hemihypertrophy, GU malforms

Genetic factors, deletion or translocations

Question 11

Syndromes Associated With WilmsTumor

Answer 11

Aniridia
Genitourinary abnormalities
Mental Retardatin

Question 12

genetic alteration of WilmsTumor

Answer 12

Deş 11p13 means WT1 PAX6

Question 13

DİAGNOSİS AND DIFFERENTIAL DİAGNOSİS of abdominal mases?

Answer 13

in a child should be considered malignant until diagnosis

Labs, renal and hepatic function

Imaging studies

CT is useful to define the extent of the disease

MRI may be helpful in defining an extensive tumor

Question 14

wilms tümörü ve Neuroblastoma hangi yaş gurubunda görülür?

Answer 14

preschool

Question 15

hepato blastoma hangi yaş gurubunda görülür

Answer 15

0 3 yaş

Question 16

wilms tümörünün klinik belirtileri nelerdir?

Answer 16

unilateral flank mass

Aniridia

Hemihypertrophy

Question 17

Neuroblastomanın klinik belirtileri nelerdir?

Answer 17

genitourinary obstruction

RACOON EYES

mycolonous opsoclonus

Dierrhea

Question 18

Hepatoblastomanın klinik belirtileri nelerdir?

Answer 18

right upper quadrant mass
JAUNDİCE
earLy PUBERİTY an males

Question 19

wilms tümörünün Lab bulguları nelerdir?

Answer 19

Elevated partial thromboplastin time

Heamatouria
Polycthemia
Thrombacytosis

Question 20

Neuroblastomanın Lab bulguları nelerdir?

Answer 20

increased
urinary vallinymandelic acid
homoulvanic acid
or ferritin

Question 21

Hepatoblastomanın lab bulguları nelerdir

Answer 21

ıncreased Alfa FETOPROTEİN

Question 22

İMP things about Treatment and Prognosis of WİLLMS Tumor

Answer 22

Surgery

Preop chemotherapy:
if intravascular spread or very large invasive tumors; if bilateral;

Considered Stage III if imaged only

Question 23

Hepatablostama hangi lobda gelişir=

Answer 23

It arises from the right lobe 3 times more often than the left and usually is unifocal.

Elevated alphafetoprotein level

Question 24

Treatment and Prognosis of Hepatablostama

Answer 24

High cure rates, with cure possible if mets are resected (> 65%)

Question 25

Treatment and Prognosis of Hepatocellular Ca

Answer 25

Difficult to resect and difficult to cure even with complete resection (<20%)

Question 26

what is the most common causes of palpable abdominal masses in infants

Answer 26

multicystic dysplastic kidneys and hydronephrosis

Question 27

Understand that urinary catecholamine excretion is increased in most patients with a …… and that tests of urine for …. and ….are appropriate screening tests for the tumor

Answer 27

Neuroblastoma
VMA
VHA

Question 28

Wilms tumor is associated with 1. …. 2. …. 3. ….4 ….

Answer 28

hemihypertrophy

aniridia,

somatic overgrowth

genitourinary abnormalities