<prompt> Von Hippel Landau Syndrome is associated with which type of renal neoplasm?Renal clear cell carcinomaAngiomvolipomaPapillary renal cell carcinomaRenal medullary carcinoma  <response>: Ans: A Renal clear cell carcinoma Ref: Campbell Walsh Urology, 10th edition. 2011, Page 1426Explanation: See table belowSite 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 featuresConventionalVon 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 TScTSc - 9 and 16TSc1TSc 2Massive retroperitoneal haemcrrhage10Positive 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 ARCDMulticentricity is an important feature 40Trisomy 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 Hales 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; FM if not associated with TSFat on CT -20HU diagnostic of AML and excludes RCCPregnancy increases risk of haemorrhage from AML 