This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Renal Tumors, Subject name: Surgery
[{'content': 'Von Hippel Landau Syndrome is associated with which type of renal neoplasm? \nSelect and explain. \nA. Renal clear cell carcinoma, B. Angiomvolipoma, C. Papillary renal cell carcinoma, D. Renal medullary carcinoma', 'role': 'user'}, {'content': "Answer: A \nExplanation: 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", 'role': 'assistant'}]