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What is the most common renal neoplasm of childhood?

Wilms tumor


What three syndromes are associated with an increased risk for wilms tumors

Denys-Drash (DDS)
- associated with inactivation of WT1 gene on 11p13

- associated with WT2 inactivation on band p15.5 of chromosome 11
- presents with hypermegaly of all organs in the body

WAGR syndrome
- associated with inactivation of WT1 gene on 11p13

**only need one mutated copy of WT1 gene**


Renal papillary adenoma

Small/discrete (<0.5cm) tumors arising from the renal tubular epithelium
- commonly found at autopsy (7-22%)

Are usually pale-yellow in appearance

*share similar IHC and cytogenic features with papillary cancers
- includes trisomy 7 and 17*

**the prognostic factor is SIZE >3cm = going to die**



Benign neoplasm consisting of smooth muscle and fat droplets

Clinical importance of angiomyolipoma is due largely to spontaneous hemorrhages.

**Are present in 25-50% of patients with tuberous sclerosis**
- caused by a loss of function mutations in TSC1/2 tumor suppressor genes
- characterized by lesions of the cerebral cortex and skin abnormalities such as unusually benign tumors



An epithelial neoplasm composed of large eosinophilic cells with having small rounded normal nuclei appearance
- accounts for roughly 5-15% of renal neoplasms

Tumors are tan/brown and homogeneous with a generalized central scar (33%)
- the scar is fibrotic more than necrosis


Renal cell carcinoma (RCC)

Represent 3% of all cancers in US
- makes up 85% of renal cancers as well
- 5% in males, 3% in females (2:1)
- usually occurs in 60-70s

Risk factors
- smokers
- hypertension
- obese patients
- cadmium exposure
- acquired polycystic disease with dialysis (30x increase)

3 common types
- clear cell
- papillary cell
- chromophobe renal

Common metastasis sites = lungs and bones


Clear cell carcinoma

Is the most common type of RCC overall
- accounts for 65% of total RCC’s
- in 40-60% of clear cell patients, they present with hundreds of bilateral renal cysts

Make of cells with very clear cytoplasm
- most common arise in the proximal tubular epithelium and appear bright yellow-gray-white with foci of hemorrhagic discoloration

*most are sporadic in nature, but those that aren’t are tied to familial forms of Von hippel-Lindau disease*
- VHL = also shows hemangioblastomas of the cerebellum and retina
- VHL pathogenesis = VHL gene mutations in 3p25 band on chromosome 3 in both copies

**very commonly invades the renal vein and grows as a solid column of cells before metastizing**


How does VHL mutations cause clear cell carcinomas?

Normal VHL protein action = degrades hypoxia-induced factors (HIF’s)

In VHL disease = HIFs stay around and are transcription factors for vascular endothelial growth factor (VEGF)


Clinical features of renal cell carcinoma

Triad of
- painless hematuria (most common 50%)
- Palpable abdominal mass
- dull flank pain
**may not show all of these three, but these are the most common**

Often shows paraneoplastic effects also
- polycythemia
- fever
- hypercalcemia
- Cushing syndrome


Papillary renal cell carcinoma

Account for 10-15% of all renal cancers and are frequently multi focal and bilateral

Look like early-stage tumors and have a unique papillary growth pattern
- have clear and pinkish cytoplasm

Can be familial and sporadic forms
- familial = MET proto-oncogene mutations on chromosome 7q
- sporadic = can be above, but also can be idiopathic


Chromophobe renal carcinoma

5% of all renal cell carcinomas

Composed of cells with prominent cell membranes and pale eosinophilic cytoplasm
- have a “halo” like appearance around the nucleus

Have multiple chromosome losses and extreme hypodiploidy genetically
- are thought to grow from intercalated cells of collecting ducts and have excellent prognosis
- also difficult sometimes to distinguish from oncocytomas


Urothelial cell carcinomas (transition cell carcinoma)

5-10% of primary tumors arise from the urothelial of the renal pelvis
- are also the majority of tumors found in the ureter
- most occur in 60-70s

Have a wide variety of ranges (can be grade 1 benign to aggressive stage 4 metastatic)
- regardless they all have poor prognosis since they have a very high tendency to infiltrate the walls of the renal pelvis and calyces

Produce hematuria and are always small when discovered (since hematuria starts very early
- in late stages = hydronephrosis


Wilms tumor


Most common in children 2-5 yrs of age

Are typically large solitary well-circumscribed masses with tan-gray colors

Can present with “nephrogenic rests” which are precursor lesions of wilms tumors in the renal parenchyma adjacent to the tumor
- if present, increases risk for development of wilms tumors in the contralateral kidney

Clincial features
- fever
- sometimes hematuria
- palpable mass that extends pass midline (most common)

Prognosis is very good and only requires surgical resection and mild chemo unless diffuse anaplasia is present


If a wilms tumor has foci of anaplasia on histology, what gene is additionally mutated?

- also signals resistance to chemotherapy

**foci of anaplasia = deep purple and segmented nuceli**


Urinary bladder cancers

More common in white males than females and other races(2x) and more common in the industrialized world
- most common patients aged 50-80s

95% are epithelial cell origin with most being urothelial or transitional tumors

Risk factors
- cigarette smoking
- occupational carcinogens
- family history
- excessive cyclophosphamide use or radiation use
- **schistosoma infections

- painless hematuria
- flank pain and sometimes hydronephrosis if chronic


Urinary bladder pathogenesis

Believed to be initiated by deletions of the tumor suppressor genes on 9p and 9q
- this leads to superficial papillary tumor formation which then leads to urinary bladder neoplasms and invasive forms when these tumors acquire TP53 mutations
- **loss of genes from chromosome 9**


What are the two precursor lesions of invasive urothelial carcinoma?

1) noninvasive papillary tumor
- most important prognostic factor is grade of the tumor
- 4 grades: (1) = papilloma, (2) = papillary urothelial neoplasm, (3) = low grade papillary urothelial carcinoma, (4) = high grade papillary urothelial carcinoma.

2) carcinoma in situ (CIS)
- are malignant-like cells with a flat urothelium
- cells are often shed into he urine where they are detected by cytology
- most important prognostic factor is stage
- without treatment, 50-75% of these cases progress to invasive cancers


Treatment of urinary bladder neoplasms

Depends on the grade and appearance of tumors
- small and low grade tumors = just resection

- high risk of recurrence in general = topical immunotherpies
*often uses weakened strains of TB toxin called (bacillus calmette- Guerin (BCG))

- tumors that are invasive or high grade = radical cystectomy and chemotherapy

Advanced stage and grade = palatative care and chemotherapy