Wilms Tumor Flashcards
(27 cards)
Most common malignant renaltumor of
childhood
Wilms tumor or Nephroblastoma
Peak age group of Wilms tumor
Peak: 3-4yrs old
The clinical syndromes associated with WILMS?
The clinical syndromes associated with
WT include WAGR syndrome (WT, Aniridia, Genitourinary mal-
formations, mental Retardation), Denys-Drash syndrome (pseu-
dohermaphroditism, mesangial sclerosis, renal failure, and
WT), and overgrowth syndromes like Beckwith-Wiedemann
syndrome (somatic gigantism, omphalocele, macroglossia,
genitourinary abnormalities, ear creases, hypoglycemia, hemihypertrophy, and a predisposition to WT and other malignan-
cies) and Simpson-Golabi-Behmel syndrome.
_____ is tumor suppressor gene at chromosome 11p13 that was
isolated from a child with WAGR syndrome.____ is likely to
play a specific role in glomerular and gonadal development
WT1
Beckwith-Wiedemann syndrome maps to chromosome 11p15.5; this locus is also referred to as ___.
WT2
Patients with ______ have higher relapse and mortality rates.
loss of heterozygosity (LOH) at 16q and 1p
The NWTS classifies all tumors as having either FH or unfavorable histology (UH).
The UH tumors include:
anaplastic tumors
clear cell sarcoma
rhabdoid tumor of kidney
WT reflects the development of the normal kidney, consisting of
three components: ______
Blastemal, epithelial (tubules), and stromal
elements, in varying proportions
The histologic feature of greatest clinical significance in WT is _____
anaplasia
_____ is the most lethal renal neoplasm in children.
Rhabdoid tumor of the kidney or RTK
classic presentation for WT is ?
abdominal swelling is discovered by the child’s mother or by a physician during a routine physical examination.
A smooth, firm, nontender mass on one side of the abdomen
is felt.
differential diagnosis of WT includes _____
other malignant
childhood lesions of the kidney, neuroblastoma, and benign
conditions such as hydronephrosis, polycystic disease, and
splenomegaly in left-sided tumors.
Excretory
urography (intravenous pyelography) was once the main-
stay of imaging in WT and now has largely been replaced by
___________ and _______
ultrasonography and computed tomography (CT) scanning
The most common sites of metastases of WT
are in the _____
lungs (80%), lymph nodes, and liver.
The NWTS has shown that although RT does not need to be
given immediately after surgery, a delay of ____ days after
surgery was associated with a significantly higher abdominal
relapse rate, particularly among patients with UH tumors.
≥10 days
In the COG protocols, the dose is ____ Gy
for most indications except for stage III DA and stages I to
III RTK, where a higher dose of ____ Gy is recommended
In the COG protocols, the dose is 10 Gy
for most indications except for stage III DA and stages I to
III RTK, where a higher dose of 19.8 Gy is recommended
____ Gy: HIGH Incidence of 54% (Washington Univ.) of scoliosis
> 30 Gy
> __ Gy flank rt has higher pregnancy outcomes in the future
25 Gy
Syndrome with WT with Highest ESRD?
Denys Drash syndrome (74)
To decrease ______, shielding of femur and acetabulum is done
Slipped femoral capital epiphysis
Inferior border of WLI is?
Costophrenic angles
In NWTS2, what drug is added in dactino and vincistine that increased 2 year survival for stage II-IV tumors to 77%
Doxorubicin
The COG staging and risk-group classification for treatment
assignment in the new generation of WT protocols This classification will, in addition to tumor stage, also consider the _____
eL-STAR
LOH at 1p and 16q Stage tumor weight patient’s age response to chemotherapy in children with FH tumors and lung metastases.
COG: ONLY those children
surgery-only therapy
