2. pediatri abdominal masses Flashcards
Most common cancer in infants nedir?
Neuroblastoma
Neuroblastoma’nın özellikleri nelerdir?
Most common cancer in infants
Genetic links/factors involved
N-myc
oncogene, chromosome deletion
whaty is Most common cancer in infants
Neuroblastoma
Average age is 18 months; 80% < 5
Clinical Presentation of neuroblastoma?
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
How to diagose neuroblastoma?
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
Neuroblastoma Staging?
- 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
Treatment and Prognosis of neuroblastoma
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;
what is High-risk neuroblastoma
long term survival rates between 25% and 35%
Highdose chemotherapy with autologous stem cel cellrescue–Surgery–Radiation
13-c / s retinoic acid ( isotretinoin , Accutane
böbrek tümörleri nelerdir?
primarly Wilms, rapidly growing vascular abdominal tumors
clear cell sarcoma
renal cell CA, lymphoma, PNET, rhabdoid
Incidence and Etiology of kidney tumors
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
Syndromes Associated With WilmsTumor
Aniridia
Genitourinary abnormalities
Mental Retardatin
genetic alteration of WilmsTumor
Deş 11p13 means WT1 PAX6
DİAGNOSİS AND DIFFERENTIAL DİAGNOSİS of abdominal mases?
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
wilms tümörü ve Neuroblastoma hangi yaş gurubunda görülür?
preschool
hepato blastoma hangi yaş gurubunda görülür
0 3 yaş
wilms tümörünün klinik belirtileri nelerdir?
unilateral flank mass
Aniridia
Hemihypertrophy
Neuroblastomanın klinik belirtileri nelerdir?
genitourinary obstruction
RACOON EYES
mycolonous opsoclonus
Dierrhea
Hepatoblastomanın klinik belirtileri nelerdir?
right upper quadrant mass
JAUNDİCE
earLy PUBERİTY an males
wilms tümörünün Lab bulguları nelerdir?
Elevated partial thromboplastin time
Heamatouria
Polycthemia
Thrombacytosis
Neuroblastomanın Lab bulguları nelerdir?
increased
urinary vallinymandelic acid
homoulvanic acid
or ferritin
Hepatoblastomanın lab bulguları nelerdir
ıncreased Alfa FETOPROTEİN
İMP things about Treatment and Prognosis of WİLLMS Tumor
Surgery
Preop chemotherapy:
if intravascular spread or very large invasive tumors; if bilateral;
Considered Stage III if imaged only
Hepatablostama hangi lobda gelişir=
It arises from the right lobe 3 times more often than the left and usually is unifocal.
Elevated alphafetoprotein level
Treatment and Prognosis of Hepatablostama
High cure rates, with cure possible if mets are resected (> 65%)
Treatment and Prognosis of Hepatocellular Ca
Difficult to resect and difficult to cure even with complete resection (<20%)
what is the most common causes of palpable abdominal masses in infants
multicystic dysplastic kidneys and hydronephrosis
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
Neuroblastoma
VMA
VHA
Wilms tumor is associated with 1. …. 2. …. 3. ….4 ….
hemihypertrophy
aniridia,
somatic overgrowth
genitourinary abnormalities