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Kidney

Renal Cysts And Cystic Disease

  • Problems with Bosniak Classification
    - Difficulty deciding between cat. 2 and cat. 3
    - Important Rx implications
    - Relatively frequent problem
    - Wall or septum too thick?
    - > 3 cm in diameter
    - Equivocal enhancement
    - Or, if there are Sx (e.g., hematuria)
    - Call these “2F” (need follow-up)
    -- Repeat scans in 3, 6, and 12 months, then annually
    - Follow for 5 years (cystic RCCs are slow growing)
  • Bosniak Category 1:
    Simple Renal Cyst
    Features:
    1. Imperceptible or hairline walls
    2. No septa, calcifications or solid components
    3. Water attenuation
    4. No enhancement
    -  < 20 HU change is probably “pseudoenhancement”
    5. Management: Ignore
  • Bosniak Category 2
    Features
    1. Few hairline-thin septa without measureable enhancement
    2. May have thin calcification in septa or wall
    3.  (Subtype): Hyperdense cyst
    - Homogeneously high attenuation (usually > 70 HU)
    -  - 3 cm diameter
    - Nonenhancing
    4. Management: Ignore
  • Cystic Renal Masses
    Category 2 – Benign, Minimally Complicated

    Hyperdense Cyst
    - Usually not due to acute bleed
    - These may have fluid – debris level
    - Same density on unenhanced and enhanced (usually 70 – 100 HU)
  • Bosniak Category 3:
    Complex Cyst or Neoplasm

    Features:
    - Thickened, irregular walls or septa with measurable enhancement
    Management:
    - Surgery for most patients
    - Observe (or even ignore) for those with serious comorbidities or limited life expectancy
  • Bosniak Category 4:
    Cystic Neoplasm

    Features:
    - Same as for Category 3, but also with enhancing soft tissue components
    Management:
    - Surgery for most
    - Nephron-sparing if small + accessible
    - Observe (or even ignore) for those with serious comorbidities or limited life expectancy
  • Multilocular Cystic Nephroma
     (Multilocular Renal Cyst)
    - Multiple non-communicating cysts
    - Surrounded by thick capsule
    - Septa enhance, may calcify
    - Herniation into renal pelvis
    - Usually boys and middle age women
    - Caution!
    - RCC can have identical appearance
  • “ The classification of renal cystic lesions suggested 25 years ago, now referred to as the Bosniak renal cyst classification, remains pertinent to the diagnosis and management of these difficult to diagnose complicated cystic masses.”
    The Bosniak Renal Cyst Classification: 25 Years Later
    Bosniak MA
    Radiology 2011; 262:781-785
  • “ Pseudoenhancement of renal cysts significantly correlates with size smaller than 1 cm and central location. Although pseudoenhancement increase with larger numbers of detectors, the correlation was not statistically significant. Cysts in the 1- to 1.5 cm range have a 19% likelihood of pseudoenhancement.”
    Pseudoenhancement of Renal Cysts: Influence of Lesion Size, Lesion Location, Slice Thickness, and Number of MDCT Detectors
    Tappouni R et al.
    AJR 2012; 198:133-137
  • “ Renal cysts are common in adults, and the prevalence increases with age to greater than 27% in patients older than 50 years.”
    Pseudoenhancement of Renal Cysts: Influence of Lesion Size, Lesion Location, Slice Thickness, and Number of MDCT Detectors
    Tappouni R et al.
    AJR 2012; 198:133-137
  • “ A less than 10 HU increase in attenuation is considered within the technical limits of the study and is not considered to represent enhancement.”
    Pseudoenhancement of Renal Cysts: Influence of Lesion Size, Lesion Location, Slice Thickness, and Number of MDCT Detectors
    Tappouni R et al.
    AJR 2012; 198:133-137
  • " Diagnostic performance in characterizing lesions as benign or malignant, however, is not statistically different for the thick and thin images."

    Characterization of Atypical Cystic Renal Masses with MDCT: Comparison of 5-mm Axial images and Thin Multiplanar Reconstructed Images
    Bertolotto M et al
    AJR 2010; 195:693-700

  • "Analysis of volume data sets is associated with less intraobserver and interobserver variability of 5-mm axial images. Wall thickness and the number and thickness of septa may differ, resulting in assignment of different Bosniak categories."

    Characterization of Atypical Cystic Renal Masses with MDCT: Comparison of 5-mm Axial images and Thin Multiplanar Reconstructed Images
    Bertolotto M et al
    AJR 2010; 195:693-700

  • Localized Renal Cystic Disease : facts
    - Conglomerate mass of multiple simple cysts of various sizes, seperated by enhancing or atrophic renal tissue
    - Cysts may involve entire kidney or portion of the kidney
    - Typically kidney will show normal excretion of contrast material
    - Benign condition which does not progress to renal failure
  • "Also known as segmental renal cystic disease and unilateral renal cystic disease, localized renal cystic disease is a rare, nonhereditary, noprogressive condition that is characterized by multiple cysts in one kidney, with no cysts in the other kidney or other organs."

    Adult Renal Cystic Disease: A Genetic, Biological, and Developmental Primer
    Katabathina VS et al.
    RadioGraphics 2010; 30:1509-1523

  • Autosomal Dominant Polycystic Renal Disease: Extrarenal Manifestations
    - Hepatic cysts
    - Pancreatic cysts
    - Seminal vesicle cysts
    - Splenic cysts
    - Intracranial arterial aneurysms
    - Aortic aneurysms
    - Abdominal wall hernias
    - Colonic diverticula
    - Aortic and mitral valve abnormalities
  • Autosomal Dominant Polycystic Renal Disease: Complications
    - Infection
    - Hemorrhage
    - Cyst rupture
    - End stage renal disease (45% of patients) develops by age 60
  • Autosomal Dominant Polycystic Renal Disease: Facts
    - Most common hereditary renal cystic disease
    - Occurs in one in 400-1000 live births
    - Affects 300,000 to 600,000 Americans
    - 3rd most common cause of end stage renal disease
    - 90% of cases are hereditary in nature
    - Mutation in gene PKD1 and PKD2
  • "Patients with autosomal dominant polycystic kidney disease have a mean renal volume of more than 1000 mL (the normal mean is 150 mL) and an age related increase in total renal volume and total cyst volume, with a mean annual increase of 63.4 mL in renal volume."

    Adult Renal Cystic Disease: A Genetic, Biological, and Developmental Primer
    Katabathina VS et al.
    RadioGraphics 2010; 30:1509-1523

  • "Renal cystic diseases in adults are a heterogenous group of disorders characterized by the presence of multiple cysts in the kidney. These diseases may be categorized as hereditary, acquired, or developmental on the basis of their pathogenesis."

    Adult Renal Cystic Disease: A Genetic, Biological, and Developmental Primer
    Katabathina VS et al.
    RadioGraphics 2010; 30:1509-1523

  • Medullary Sponge Kidney: Facts
    - Congenital developmental abnormality characterized by ectasia and cystic dilatation of the intrapyramidal or intrapapillary portions of the renal medullary collecting ducts
    - Occurs in 1 in 5,000 patients
    - Usually sporadic cases
    - Usually detected incidentally although some patients present with renal colic, hematuria, and urinary tract infections
  • Select Renal Cystic Diseases in Adults

    Hereditary disease

    - Autosomal dominant polycystic kidney disease
    - Medullary cystic renal disease
    - Von Hippel-Lindau disease
    - Tuberous sclerosis complex

    Nonhereditary disease
    - Acquired cystic kidney disease
    - Medullary sponge kidney
    - Multicystic dysplastic kidney
    - Localized renal cystic disease 

  • Polycystic Kidney Disease: Differential Dx (in theory)
    - Multiple simple cysts
    - Von Hippel-Lindau disease
    - Acquired uremic cystic disease
    - Infantile PCKD
  • Polycystic Kidney Disease: Clinical Presentation
    - Hypertension
    - Azotemia
    - Hematuria
    - Proteinuria
    - Abdominal or back pain
  • Polycystic Kidney Disease: Facts
    - Autosomal dominant with nearly a 100% penetrance
    - Defect on short arm of chromosome 16
    - Cysts also occur in liver (up to 80% of cases), and pancreas (up to 9% of cases)
    - Patients may also have an increased incidence of cerebral aneurysms
  • Cystic Renal Masses
    - Cystic clear cell carcinoma
    - Multilocular cystic renal cell carcinoma
    - Cystic Nephroma
    - Mixed Epithelial and Stromal tumors (MEST)
    - Complex benign cystic renal lesions
  • "Because cystic renal cell carcinomas, benign complicated cysts, and other cystic tumors can be in distinguishable at gross pathologic inspection, and a definitive diagnosis can require a histologic examination, an overlap in imaging findings is not unexpected."

    Clinical and Radiologic Features of Cystic Renal Masses
    Freire M, Remer EM
    AJR 2009; 192:1367-1372

  • "Given the distribution of cyst and tumor attenuation values and lesion heterogeneity, a homogeneous mass measuring 70 HU or greater at unenhanced CT has a greater than 99.9% chance of representing a high-attenuation renal cyst."

    Can High-Attenuation Renal Cysts Be Differentiated from Renal Cell Carcinoma at Unenhance CT?
    Jonisch AI et al.
    Radiology 2007; 243:445-450
  • "The findings from this study may help differentiate high attenuation renal cysts from renal cell carcinomas at unenhanced CT and may suggest the next appropriate imaging study for definitive characterization."

    Can High-Attenuation Renal Cysts Be Differentiated from Renal Cell Carcinoma at Unenhance CT?
    Jonisch AI et al.
    Radiology 2007; 243:445-450
  • "Greater than 95% of high attenuation renal cysts are homogeneous in attenuation."

    Can High-Attenuation Renal Cysts Be Differentiated from Renal Cell Carcinoma at Unenhance CT?
    Jonisch AI et al.
    Radiology 2007; 243:445-450
  • "A homogeneous renal mass measuring greater than 70 HU at unenhanced CT has a greater than 99.9% chance of representing a high attenuation renal cyst rather than a renal cell carcinoma."

    Can High-Attenuation Renal Cysts Be Differentiated from Renal Cell Carcinoma at Unenhance CT?
    Jonisch AI et al.
    Radiology 2007; 243:445-450
  • What is a Bosniak IIF?

    - Cysts with multiple septae with minimal thickening or calcification which is thick and nodular or intrarenal non-enhancing high density (>3cm) lesion
    - These lesions are felt to be benign and routine follow-up advised
  • The Bosniak Renal Cyst Classification System

    - Category I
    - Category II
    - Category IIF
    - Category III
    - Category IV
  • von Hippel-Lindau Disease: Renal Pathology

    - Renal cysts- occur in 50-75% of patients and are usually multiple and bilateral
    - Renal cell carcinoma-occur in 28-45% of patients and occur at a younger age (30-36 yrs). The lesions are often multiple and bilateral and may be hypovascular or cystic lesions with mural nodules
  • von Hippel-Lindau Disease: Adrenal Pathology

    - Pheochromocytoma
    - Occur in up to 30% of families with VHL
    - They are bilateral in up to 50% of patients with a malignancy rate of around 10%
    - Up to 18% are extraadrenal in location
  • "On portal venous phase contrast enhanced CT scans, attenuation greater than 70HU or moderate or marked internal heterogeneity favor a diagnosis of renal cell carcinoma oer a diagnosis of high attenuation renal cyst."

    Distinction of Renal Cell carcinomas from High Attenuation Renal Cysts at Portal Venous Phase Contrast Enhanced CT
    Suh M et al
    Radiology 2003; 228:330-334
  • Sickle Cell Disease:Organ Involement

    - Bone
    - Brain
    - Lungs
    - Liver
    - Spleen
    - Kidney
  • Sickle Cell Disease: Subgroups

    - SS disease (homozygous Hb SS)
    - SC disease
    - S-thal
  • Sickle Cell trait is associated with a rare renal tumor- medullary carcinoma.