Renal Tumors Overview and Classification

Abdominal pain = 20 - 30% of cases Hematuria = 20 - 30% of cases Hypertension = 20 - 30% of cases Anemia = 20 - 30% of cases

WAGR Syndrome = Aniridia and genital abnormality Denys-Drash Syndrome = Gonadal dysgenesis and renal abnormality Beckwith-Wiedemann Syndrome = Macroglossia and hemihypertrophy None = Enlargement of adrenal cortical gland

Oncocytic and chromophobe renal tumors = Oncocytoma Clear cell renal tumors = Clear cell renal cell carcinoma Embryonal neoplasms of the kidney = Nephroblastoma (Wilms tumor) None = Renal epithelial neoplasm

<p>Blastemal = Undifferentiated cells Epithelial = Tumor differentiation Stromal = Supportive tissue None = Vascular component</p> Signup and view all the answers

<p>90% = Excellent long-term survival 70% = Good long-term survival 50% = Moderate long-term survival 30% = Poor long-term survival</p> Signup and view all the answers

<p>Lungs = More than 50% Bones = 33% Regional lymph nodes = Followed in order Liver = Followed in order</p> Signup and view all the answers

<p>Clear cell carcinoma = Cancer cells with clear cytoplasm containing glycogen PAX8 and PAX2 = Positive stains for renal cell carcinoma CK20 = Negative stains for renal cell carcinoma Vascular network = Delicate network interspersed among clear cells</p> Signup and view all the answers

<p>Age of occurrence = 3 - 4 years Epidemiology = More common in Africans Genetic alterations = WT1 gene at chromosome 11p13 Clinical presentation = Abdominal mass with no associated symptoms</p> Signup and view all the answers

<p>Renal cell carcinoma = 70% to 80% of cases Nephroblastoma = Malignant embryonal tumor Clear cell RCC = Nests of clear cells Familial Wilms tumor = Slight female preponderance</p> Signup and view all the answers

<p>Well circumscribed lesion = Usually no pseudocapsule Oncocytes = Large round eosinophilic cells with dense granular cytoplasm Nuclei = Round and regular with even chromatin Stroma = Edematous and hyalinized fibrous stroma</p> Signup and view all the answers

<p>Osteolytic lesion = Result of bone metastasis Alkaline phosphatase = Marker for bone metastasis Cystic change = Often present in renal cell carcinoma Hemorrhage = Possible gross pathology feature</p> Signup and view all the answers

<p>Positive: CAIX = Diffuse, membranous in 75 - 100% Positive: CD10 = Proximal tubular marker Negative: Inhibin = Not associated with clear cell RCC Negative: TTF1 = Not associated with clear cell RCC</p> Signup and view all the answers

<p>Hematuria = Intermittent and may be microscopic Flank pain = Pain in the side and back Flank lump = Visible mass in the flank area Paraneoplastic syndromes = Systemic symptoms not directly related to the kidney</p> Signup and view all the answers

<p>Relapses = ~15% within 2 years Congenital tumors = Very rare Familial cases = 1 - 2% Syndromic association = 10 - 15% of cases</p> Signup and view all the answers

<p>Cigarette smoking = Most significant risk factor Obesity = Particularly in women Exposure to asbestos = Environmental risk factor Acquired polycystic kidney disease = Secondary to dialysis</p> Signup and view all the answers

<p>CD117/KIT = Cell surface receptor E-cadherin = Cell adhesion molecule S100A1 = Calcium-binding protein PAX8 = Transcription factor</p> Signup and view all the answers

<p>Clear cell renal tumors = Most common malignant tumor in adults Nephroblastoma (Wilms tumor) = Malignant tumor primarily affecting children Renal oncocytoma = Benign tumor with distinctive mitochondrial mutations Papillary renal tumors = Tumors characterized by papillary structures</p> Signup and view all the answers

<p>Wilms tumor = Primarily occurs in children Renal cell carcinoma history = More common in elderly Familial Renal carcinoma = More likely to be bilateral Nephrogenic rests = Thought to develop from persistent tissue</p> Signup and view all the answers

<p>Silent presentation = Only 10% show classic symptoms Metastasis = Can occur widely before local symptoms arise Age group = Sixth and seventh decades of life Male preponderance = Ratio of 2:1</p> Signup and view all the answers

<p>Mixed epithelial and stromal renal tumors = Renal tumors consisting of both epithelium and stroma Metanephric tumors = Tumors arising from embryonic renal tissue Renal mesenchymal tumors = Tumors originating from renal connective tissue Embryonal neoplasms of the kidney = Tumors arising from embryonic precursor cells</p> Signup and view all the answers

<p>Renal cell carcinoma = Most common malignant renal tumor in adults Nephroblastoma = Tumor observed predominantly in children Renal oncocytoma = Generally benign and asymptomatic Collecting duct tumors = Rare tumors with varying clinical implications</p> Signup and view all the answers

<p>Polycythemia = Increased hematocrit &gt; 60% Hypercalcemia = Increased calcium due to PTH-like hormone Cushing syndrome = Hormonal imbalance leading to body changes Amyloidosis = Deposits of amyloid in tissues</p> Signup and view all the answers

<p>Oncocytoma = Characterized by respiration-defective mitochondria Clear cell renal cell carcinoma = Associated with aneuploidy losses of chromosome 1 Chromophobe renal cell carcinoma = May progress from certain oncocytomas Papillary renal tumors = Tumors arising from distinct genetic pathways</p> Signup and view all the answers

<p>Occurrence rate = 1% to 3% of all visceral cancers Renal malignancy percentage = 85% of renal malignancy in adults Sporadic versus familial = Most cases are sporadic Risk factor gender = More common in males than females</p> Signup and view all the answers

<p>Well-circumscribed = Clearly defined borders Mahogany-brown cut surface = Distinctive color when sectioned Central scar = Area observed in imaging studies M:F ratio of 2:1 = Higher prevalence in males</p> Signup and view all the answers

<p>Renal oncocytoma = 5-9% of renal tumors Clear cell renal carcinoma = The most common type of renal cancer Nephroblastoma = Common in pediatric patients Papillary renal tumors = Not specified but less frequent than clear cell carcinoma</p> Signup and view all the answers

<p>Renal oncocytoma = Often asymptomatic, may cause flank pain Clear cell carcinoma = Commonly diagnosed with systemic symptoms Nephroblastoma = May present as a palpable abdominal mass Collecting duct tumors = Often diagnosed late due to insidious onset</p> Signup and view all the answers

<p>Aneuploidy = Associated with certain oncocytomas CCDN1 rearrangements = Linked to the pathogenesis of oncocytomas Chromosome Y loss = Common in tumors with aneuploidy p53 mutations = May facilitate progression to chromophobe carcinoma</p> Signup and view all the answers