Renal neoplasms Flashcards
Benign kidney tumors
Renal papillary adenoma
Angiomyolipoma
Oncocytoma
Malignant kidney tumors
Renal cell carcinoma
Transitional cell carcinoma of renal pelvis
Wilms tumor/nephroblastoma
Adrenal neuroblastoma
Benign kidney tumor that grossly appears as small cortical pale yellow-gray nodules (<0.5 cm in diameter) that arises from tubular epithelium
Renal papillary adenoma
Microscopy of kidney lesion shows complex, branching papillary process
Renal papillary adenoma or renal cell adenocarcinoma –> hard to differentiate
Benign tumor of kidney with blood vessels, smooth muscle, and fat. Present in 25-50% of tuberous sclerosis due to loss of function in tumor suppressor gene, TSC1 and TSC2
Angiomyolipoma
Syndrome characterized by hamartomatous lesions in the cerebral cortex causing epilepsy and mental retardation, skin lesions (Shagreen patches and ash leaf patches), and other benign tumors (like rhabdomyoma of the heart).
Tuberous sclerosis
Benign renal tumor that is clinically important due to tendency to bleed spontaneously
Angiomyolipoma
Gross appearance of renal angiomyolipoma
Large, yellowish, and firm tumor
Gross morphology of renal oncocytoma
Tan or mahogany brownish colored lesion with central scar
Benign kidney tumor that arises from intercalated cells of collecting ducts. Large pink cells on microscopy. Electron microscopy shows cells rich in mitochondria.
Oncocytoma
Description of oncocytes
Large cells with are very pink
Exposure that has a strong association with renal cell carcinoma
Cigarette smoking
Familial variants (4%) of renal cell carcinoma
Von Hippel-Lindau disease
Hereditary/familial clear cell carcinoma
Hereditary papillary carcinoma
Normal function of VHL gene
Downregulates hypoxia inducing factor-1 –> decreased production of VEGF, PDGF, and IGF-1
Result of abnormal VHL gene in Von Hippel-Lindau disease
Increased cell growth and angiogenesis
Manifestations of Von Hippel-Lindau disease not seen in hereditary/familial clear cell carcinoma
Hemangioblastomas of cerebellum, cysts, and multiple RCC, other than renal
Mutation in hereditary papillary carcinoma
MET proto-oncogene resulting in multiple bilateral kidney tumors
Inheritance of hereditary papillary carcinoma
Autosomal dominant
Gene involved in 98% of all clear cell carcinomas, both familial and sporadic
VHL gene on chromosome 3p
What is also regulated by VHL gene, other than HIF-1?
Expression of EPO
Classifications of renal cell carcinoma
Clear cell carcinoma
Papillary carcinoma
Chromophobe renal cell carcinoma (great prognosis)
Collecting/Bellini duct carcinoma
Renal tumor with a propensity to invade the renal V to spread to IVC and right heart
Renal cell carcinoma
Spherical, bright yellow or gray-white, kidney lesion with sharply defined margins on upper or lower pole. Accompanied by hemorrhage and necrosis.
Renal cell carcinoma
Microscopic features of clear cell renal carcinoma
Clear cells in nests, cords, and sheets with PAS positive cytoplasm filled with lipid and glycogen and delicate branching vasculature.
Microscopic features of renal papillary carcinoma
Papillae with foam cells in the fibrovascular cores
Psammoma bodies may also be present
Classic triad of clinical features of renal cell carcinoma seen in 10%
Costovertebral pain
Palpable mass
Hematuria
Paraneoplastic syndromes associated with renal cell carcinoma
Polycythemia
Hypercalcemia
HTN
Hepatic dysfunction
Cushing syndrome
Eosinophilia
Leukemoid reaction
Amyloidosis
Feminization or masculinization
Associated with increased incidence of transitional cell carcinoma of renal pelvis
Balkan nephropathy
Analgesic nephropathy
Papillary, nodular, or flat kidney lesion. Microscopy shows delicate papillae lined by malignant urothelial cells (>7 thick). May be low or high grade depending on mitosis, polarity, or pleomorphism.
Transitional cell carcinoma of renal pelvis
Why does TCC of renal pelvis become symptomatic early?
Due to its location
Symptoms of TCC of renal pelvis
Hematuria
Obstruction causing palpable hydronephrosis and flank pain
Concomitant tumor seen with TCC of renal pelvis
Bladder tumor
Four groups of congenital malformations associated with Wilms tumor
WAGR syndrome
Denys-Drash syndrome
Beckwith-Weidemann syndrome
Mutations in gene encoding beta-catenin
Syndrome associated with germline deletion of 11p13 (WT1)
WAGR syndrome
Features of WAGR syndrome
Wilms tumor
Aniridia
Genital abnormalities
Mental retardation
Mutation associated with Denys-Drash syndrome
Germline abnormalities in WT1 affecting DNA-binding properties
Features of Denys-Drash syndrome
Gonadal dysgenesis
Early onset nephropathy –> diffuse mesangial sclerosis
Syndrome associated with Wilms tumor and increased risk for gonadoblastoma
Denys-Drash syndrome
Protein critical for normal renal and gonadal development that is associated with Wilms tumor when mutated
WT1
Syndrome associated with non-classical example of tumorigenesis involving WT2 on chromosome 11p15.5
Beckwith-Weidemann syndrome
Characteristics of Beckwith-Weidemann syndrome
Organomegaly
Macroglossia
Risk for other tumors
Pathway affected by beta-catenin mutation in Wilms tumor
WNT pathway
Precursor lesion of Wilms tumor
Nephroblastoma
Nephrogenic rests that share genetic alterations with Wilms tumor and are considered pre-neoplastic. When present, increase risk of development of Wilms tumor in contralateral kidney
Nephroblastoma
Large, solitary, well-circumscribed renal mass that is soft and tan-gray, with a foci of hemorrhage and necrosis. May also have cysts.
Wilms tumor
Classic triphasic microscopic features of Wilms tumor
Epithelial - abortive glomeruli and tubules
Blastemal - small blue cells
Stromal - spindle cells and myxoid areas
Describe anaplasia, sometimes seen in Wilms tumor
Large hyperchromatic pleomorphic nuclei with abnormal mitosis due to p53 mutation
Significance of presence of anaplasia in Wilms tumor
Resistant to chemotherapy
Features associated with poor prognosis in Wilms tumor
Anaplasia
Loss of genetic material on chromosomes 11q and 16q
Gain in chromosome 1q
Malignancies with increased risk after radiation for Wilms tumor
Bone and soft tissue sarcomas
Leukemias and lymphomas
Breast cancer
Tumor composed of small, primitive appearing cells that have dark nuclei and scant cytoplasm, with poorly defined cell borders growing in sheets with pleomorphism. Background is often faintly eosinophilic fibrillar material. Arranged in Homer-Wright pseudorosettes
Adrenal neuroblastoma
Metabolites in the synthesis of epinephrine that are often elevated in urine with neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma.
VMA and HVA
What is a sign of poor prognosis in adrenal neuroblastoma?
Absence of VMA production
