Angiomyolipoma of the kidney is a tumor composed of an admixture of ?
smooth muscle, blood vessels and fatty tissue
Angiomyolipoma of the kidney frequently seen associated with
tuberous sclerosis, cardiac rhabdomyoma, skin hypopigmentation
The common benign renal neoplasms are ?
(1) is the biggest risk factor for renal cancer
- Tobacco smoking
Risk factors for renal cancer besides tobacco smoking
exposure to heavy metals, a history of long-term dialysis(acquired cystic disease).
(1) accounts for 80% of renal cancers.
- Clear cell renal cell carcinoma
Both familial and sporadic types of clear cell renal cell carcinoma are associated with (1) of function of the (2) gene on chromosome (3)
- VHL tumor suppressor
Clinical features of renal cell carcinoma are (1). Constitutional symptoms of (2) are often also present.
- costovertebral angle pain, presence of a palpable mass and often hematuria (intermittent)
- fever, malaise and weight loss
Renal cell carcinomas can produce a diverse number of symptoms from abnormal hormone production such as (1) which may confuse the diagnosis.
- polycythemia, HTN, hypercalcemia and Cushing’s syndrome
Frequent metastatic sites of renal cell carcinomas are ?
lungs and bone
Tumor extension to involve perirenal or the presence of tumor within (1) are signs of poor prognosis
- renal veins (renal vein invasion)
Urothelial carcinoma originates from the (1). (2) is a frequent presenting symptom. Larger tumors may produce (3)
- urothelial lining cells of the renal pelvis
- hydro nephrosis
Urothelial carcinoma may frequently be present in other portions of the (1)
renal pelvis, ureters or bladder
(1) is the most common primary kidney tumor in children (1 in 10,000 children) and the fourth most common pediatric malignancy. Tumors are bilateral in up to 10% of cases.
- Wilms tumor or nephroblastoma
Wager syndrome patients have _____
aniridia, genital abnormalities, mental retardation
Wager syndrome features germline deletions of (1) and studies of these patients led to the identification of the (2).
2. Wilms tumor associated gene (WT1)
Denys-Drash syndrome patients have ______
- gonadal dysgenesis
Beskwith-Wiedmann syndrome patients (1) also have an increased incidence of Wilms tumors.
- enlargement of individual body parts or body segments
Tumor suppressor gene
often come to light due to presence of an easily palpated very large abdominal mass that may even extend into the pelvic area
Features of VHL -
bilateral renal cell carcinoma, family history
Diverse symptoms from hormone production (systemic symptoms unrelated to the kidney itself) Polycythemia HTN Hypercalcemia Cushing’s syndrome
Renal cell carcinoma clinical features
aniridia, genital abnormalities, mental retardation‐33% lifetime risk
gonadal dysgenesis, renal abnormalities‐90% lifetime risk
Triphasic combination of elements in WILMS tumor
1, 2, 3
epithelial cell types(abortive tubules or glomeruli)