2004 Texas Radiological Society UNKNOWN CASES Genitourinary

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2004 Texas Radiological SocietyUNKNOWN
CASESGenitourinary

• Justin Q. Ly, MD
• Douglas P. Beall, MD
• Wilford Hall Medical Center
• San Antonio, TX

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GENITOURINARY

• 40-year-old female
• Difficult to control hypertension
• Ultrasound obtained to evaluate for renal artery
  stenosis---showed incidental finding of
  indeterminate right renal cystic lesion

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2004 Texas Radiological SocietyDiagnosis
forGenitourinary Case

• Justin Q. Ly, MD
• Douglas P. Beall, MD
• Wilford Hall Medical Center
• San Antonio, TX

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Imaging Findings

• B/l Pheos
• Islet Cell Tumors of the Pancreas with Liver Mets
• Renal Cystic Lesions
• Cerebellar Hemangioblastoma

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Von Hippel Lindau (VHL) Disease

• Inherited as autosomal dominant trait with very
  high penetrance
• Genetic test 99 accurate
• Freq 135K-40K
• Target Organs
• CNS, Retina hemangioblastomas
• Kidney cysts, cancer
• Pancreas cysts, neuroendocrine/islet cell
  tumors
• Adrenal pheo
• Epididymis/Broad ligament cystadenomas
• Multiple b/l renal cysts and cystic and solid
  neoplasms most common mnaifestation of VHL
• RCC-24-45, often multifocal and b/l

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VHL

• Hemangioblastomas 50
• Cerebellum, brainstem, cord, retinal
• Mult hemangioblastomasdiagnostic
• Entire CNS (cord/brain) must be imaged
• Most pts with solitary hemangioblastoma do not
  have VHL

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Von Hippel Lindau (VHL) Disease

• WorkUp
• MRI head/spine w/Gado
• Abd CT w/contrast
• Serum catecholamines
• Ophthalmalogic exam
• Audiology
• Mutation analysis
• Family screening permits timely genetic
  counseling and early identification of
  potentially life-threatening lesions
• Abd CT in late 2nd decade

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2004 Texas Radiological SocietyUNKNOWN CASES

• Justin Q. Ly, MD
• Douglas P. Beall, MD
• Wilford Hall Medical Center
• San Antonio, TX

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