Von Hippel Landau Syndrome is associated with which type of renal neoplasm?
Correct Answer: Renal clear cell carcinoma
Description: Ans: A (Renal clear cell carcinoma) Ref: Campbell Walsh Urology, 10th edition. 2011, Page 1426Explanation: (See table below)Site of origin:Proximal tubule-Conventional (clear cell) RCC and papillary RCCDistal tubule - OncocytomasCollecting duct - Chromophobic and collecting duct RCCCalyceal epithelium near renal papillae - Renal medullary carcinomaConventional (clear cell) RCC:70-80% of RCCsWorse prognosisAggressiveRenal tumorSyndromeChromosomeGeneOther featuresConventional/Von Hippel3p25-26VHLClear cell RCCHemangioblastomas of thecentral nervous systemRetinal angiomas Pheochromocytomaclear cell RCCLindau genePapillary RCC type 1Hereditary papillary RCC7q31c-MET proto- oncogeneType 1 PRCCPapillary RCC type 2FamilialLeiomyomatosis and RCC1q42FumaratehydrataseType 2 PRCCCutaneous and uterineleiomyomasChromophobeRCCBirt-Hogg-Dube17p12q11BHD1geneChromophobe RCCOncocytomaTransitional (hybrid oncocytic) tumorsOccasional clear cell RCCCutaneous fibrofolliculomasLung cystsSpontaneous pneumothoraxOncocytoma 1p-Loss ofY, 14q Rearrang-ements of 11ql3 Spoke-wh eel pattern on angiographyStellate scar on cross-sectional imagingAbundance of mitochondriaAngiomyolipomaWunderlich Tuberous Sclerosis (TSc)TSc - 9 and 16TSc1TSc 2Massive retroperitoneal haemcrrhage(10%)Positive for HMB45Fat (plain CT: <-20 HU within a renal lesion) - diagnostic hallmarkHypervascular3p deletion. VHL gene mutation. Loss of 8p. 9p. 14q and Gain of 5qRespond to immunotherapyChromophilic (papillary) RCC:10-15% of RCCsHvpovascularSeen in End-Stage Renal Disease (ESRD) and Acquired Renal Cystic Disease (ARCD)Multicentricity is an important feature {40%)Trisomy 7.17. Loss of Y chromosomeType 1: BasophilicType 2: Eosinophilic - aggressiveChromophobic RCC:3-5% of RCCsPrognosis better than conventional RCCHypo vascularMircovesides on electron microscopy - defining featurePlant cell appearancePerinuclear haloPositive for Hale's colloidal iron (indicates presence of mucopolysaccharide) - unique to RCCPositive for cytokeratin; Negative for vimentinLoss of 1.6.8.11,18.21 .Y; VHL mutations and chromosome 3 abnormalitiesCollecting duct (Bellini duct) RCC:1% of RCCsPoor prognosisInfiltrativeAffinity for UlexeuropaceouslectmRenal medullary' carcinoma:Exclusively associated with sickle cell traitInfiltrativeRelatively hypoxic environment plays a role in fumori gene sisOncocytoma:Histology and mean size (4-6 cm) similar to Chromophobe RCCIncreased uptake on Technetium Sestamibi scan - uniqueNumerous large mitochondriaCentral scarLow gradeAngiomyolipoma (AML):Mature adipose tissue, smooth muscle and thick walled vessels seenHMB 45 positiveHyperechoic on ultrasonogramSeen in tuberous sclerosis: 30 years. F:M -- 2:1; multicentric5-6th decade; F>>M (if not associated with TS)Fat on CT (< -20HU) diagnostic of AML and excludes RCCPregnancy increases risk of haemorrhage from AML
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