Chapter 20: Related Neoplasia Flashcards
Which 3 ethnicities are most commonly affected by Wilms Tumor?
Asians > Whites > Blacks
How is Wilms tumor most often discovered?
- As large abdominal mass by parent or doc
- May exhibit pain, microscopic hematuria, and HTN
Why is the presence of nephrogenic rests in a child a significant finding?
- Putative precursor lesions of Wilms Tumors
- 25-40% of unilateral
- 100% of bilateral arise from nephrogenic rests!
Between what ages is peak prevalence for Wilms Tumor?
2-5 yo
Which 3 groups of congenital malformation/familial syndromes have associated Wilms Tumor?
- WAGR –> Wilms tumor + Aniridia + Genital anomalies + Retardation
- Denys-Drash Syndrome (90% have Wilms)
- Beckwith-Wiedemann Syndrome
What is the most common way that Wilms Tumor is acquired?
90% are SPORADIC arising in previously healthy children w/ no congenital abnormalities
Which 2 congenital malformation syndromes associated with Wilms Tumors have a strong association with the WT1 gene?
- WAGR syndrome
- Denys-Drash syndrome
What is the classic histomorphology/pattern of Wilms Tumor?
- Classic triphasic combination of:
1) Blastemal cells
2) Stromal cells
3) Epithelial cells
*Mimics the germinal development of the normal kindey
Anaplasia in the histomorphology of Wilms Tumor tends to be associated with what mutations?
Resistance to?
p53 mutations w/ resistance to chemotherapy
What is the most common genitourinary malformation seen in affected males and females with WAGR syndrome?
- Males = undescended testes
- Females = streak gonads or uterine malformations
What is the characteristic glomerular lesion seen in patients with Denys-Drash syndrome?
Diffuse mesangial sclerosis
In addition to Wilms tumors, patients with Denys-Drash syndrome are at an increased risk for what other tumor?
Gonadoblastoma (germ cell tumor)
Beckwith-Wiedemann syndrome is associated with mutations of which chromosomal region and with what associated gene?
WT2 associated w/ IGF2
Beckwith-Wiedemann syndrome is characterized by what classic findings?
- Enlargement of body organs (organomegaly)
- Macroglossia
- Hemihypertrophy
- Omphalocele
- Adrenal cytomegaly
What is the characteristic gross morphology of Wilms Tumors?
- Well-circumscribed margins
- Tan-to-gray color w/ foci of hemorrhage, cysts, and necrosis
- Large, expansile tumor in lower pole of kidney