Neoplasms of the kidney Dr. sara albeily Mbbs . Md. University of Khartoum
Neoplasms of the kidney Childhood neoplasms: * Wilms tumor ( malignant ) Adult neoplasms: * Benign tumors. * Malignant tumors.
Neoplasms of the kidney- Childhood Wilms tumor ( Nephroblastoma ): It is a malignant tumor. The most common primary renal tumor in children, and the fourth most common pediatric malignancy. Age: 2- 5 years 90% of cases are unilateral, 5-10% of cases are bilateral. 90% of cases are curable.
Wilms tumor-cont. Pathogenesis: WT1(chromosome 11) and WT2 genes. Wilms tumor can occur as a component of syndrome: 1- WAGR syndrome: W ilms tumor, A niridia , G enital anomalies and M ental retardation. 2- Denys- Drash syndrome: Wilms tumor and gonadoblastoma . 3- Beckwith- Wiedemann syndrome: organomegaly and Wilms tumor.
Wilms tumor- C linical features : - Pt present with a large abdominal mass . - usually unilateral. - may present with hematuria, abdominal pain, intestinal obstruction or hypertension.
Wilms tumor- Morphology: Grossly: Usually large, solitary, well circumscribed mass. In cut section: soft, homogenous, tan to gray surface with foci of hemorrhage, cyst formation and necrosis.
Wilms tumor- Morphology- cont. Microscopy : It is a triphasic tumor: Triphasic components: blastemal(small blue cells), stromal( fibrocytic or myxoid ) and epithelial cells(abortive tubules or glomeruli).
Benign kidney tumor- Adult 1- Papillary adenoma: Grossly: it is small tumor (less than 0.5 cm). Micro: well circumscribed papillomatous tumor composed of cuboidal cells with small regular nuclei without atypia.
Benign kidney tumor- Adult 2- Angiomyolipoma : Benign tumor composed of :blood vessels, smooth muscle and fat. Found in 25%- 50% of patients of tuberous sclerosis. Risk for spontaneous hemorrhage.
Benign kidney tumor- Adult 3- Oncocytoma : - 5- 10% of renal neoplasm. - Grossly: well encapsulated tumor, mahogany in color, with central scar in third of cases. - Micro: tumor composed of large eosinophilic cells with small benign appearing nuclei and large nucleoli.
Malignant kidney tumor- Adult Renal cell carcinoma: - represent about 3% of newly diagnosed cancer in US. - 65.000 new cases per year, and 13000 deaths from the disease. - 6-7 th decades of life - male:female = 2:1. - Tobacco is the most significant risk factor.
Malignant kidney tumor- Adult Major types of renal cell carcinoma: 1- Clear cell carcinoma: It is the most common type of renal carcinoma, 70-80% of renal cancer. Pathogenesis: deletion/mutation of VHL in chromosome 3. Usually solitary unilateral lesion. Grossly: spherical yellow to white tumor. Micro: tumor composed of cells arranged in solid or trabecular pattern, the cells have polygonal shape and abundant clear or granular cytoplasm. Clear cell carcinoma has tendency to invade the renal vein.
Clear cell carcinoma- Grossly
Clear cell carcinoma- Micro
Malignant kidney tumor- Adult Renal cell carcinoma-cont . 2- Papillary carcinoma: - 10- 15% of renal cell cancers. - familial or sporadic. - Pathogenesis: trisomies 7 and 17 and loss of Y in male patient. Grossly: can be multifocal and bilateral lesions. typically cystic and hemorrhagic. Micro: composed of cuboidal to low columnar cells arranged in papillary formation. Interstitial foam cells are common the papillary cores. Psammoma bodies may be seen.
Malignant kidney tumor- Adult Microscopy of papillary carcinoma
Malignant kidney tumor- Adult Renal cell carcinoma- cont. 3- Chromophobe carcinoma: - 5% of renal cell cancers. - has excellent prognosis compared with that of clear cell and papillary cancers. Microscopy: Tumor composed of pale eosinophilic cells with perinuclear halo arranged in solid sheets.
Malignant kidney tumor- Adult Micro of Chromophobe tumor
Malignant kidney tumor- Adult Renal cell carcinoma- cont. Clinical features: - the classical features are costovertebral pain, palpable mass and hematuria. - pt ma complain of fever, malaise, weakness and wt loss. - the most common location of metastasis is the lung. Prognosis: Influenced by multiple factors: (tumor size, infiltrative margins, histological type and tumor stage) Management: radical nephrectomy +/- chemotherapy.
Malignant kidney tumor- Adult Urothelial Carcinoma of renal pelvis - 5 to 10% of primary renal tumors. - occur in renal pelvis. - usually discovered early, pt present with hematuria (fragmentation), or flank pain(urine outflow obstruction). Microscopy: Has the same microscopy of lower urinary tract transitional microscopy. Associated with ureter and urinary bladder carcinomas.
Tumors of the urinary bladder-cont. Predisposing factors: 1- Urinary Bilharziasis . 2- Villous papilloma. 3- Cigarette smoking. 4- prolonged high doses of analgesics.
Tumors of the urinary bladder and collecting system The entire urinary collecting system from renal pelvis to urethra is lined with transitional epithelium , so its epithelial tumors assume similar morphologic pattern.
Tumors of the urinary bladder and collecting system- cont. These tumors are classified into: 1- Benign papilloma. 2- Group of papillary urothelial neoplasms of low malignant potential. 3- Urothelial carcinoma (low and high grade). 4- In situ stage of bladder carcinoma.
Clinical course: Painless hematuria is the dominant clinical presentation of all these tumors. They affect men about three times as frequently as women and usually develop between the age of 50- 70 years.
Prognosis: In general, with low grade lesions, the prognosis after removal is good, but when deep penetration of the bladder wall has occurred, the 5- year survival is less than 20%.