GENITOURINARY IMAGING 2 Flashcards
KIDNEY ANATOMY
Does US over or under estimate renal length?
Ultrasound (US) often underestimates the true renal length because of technical difficulties in imaging the entire kidney.
19F Hypertensive
kypokalaemia
elevated aldosterone
Juxtaglomerular Tumor (Reninoma)
- Secretion of renin causes HTN, hypernatremia, and hypokalemia (secondary aldosteronism).
- Most patients undergo angiography as part of the workup for HTN.
- Tumors appear as small hypovascular masses. Rare.
Reninoma is a tumour of the cells of the juxtaglomerular apparatus that produces excessive amounts of renin, resulting in a secondary form of hyperaldosteronism, manifesting clinically with hypertension (HT) and hypokalaemia1. This renal tumour is predominant in females, with a peak incidence between the second and third decade of life and, although uncommon, it is a treatable cause of HT2. It should be suspected in any patient with refractory HT associated with hypokalaemia and high levels of aldosterone.
We present the first case of reninoma reported in Argentina in a young woman with refractory HT and hypokalaemia.
In 1967, Robertson et al.6 reported the first renin-producing tumour. Kihara et al. later called them “juxtaglomerular cell tumours”7. These tumours are very uncommon, with their incidence peaking between the second and third decade of life and they are predominantly found in females, originating in the myoendocrine cells of the renal juxtaglomerular apparatus, although the production of renin by different tumours has also been demonstrated, such as Wilms tumours, carcinoid tumours, renal oncocytoma and renal cell carcinoma8,9. In accordance with the presence or absence of symptoms, JCT may occur as: a “typical” variant in the majority of cases and manifests with HT, hypokalaemia, high plasma renin and secondary hyperaldosteronism; an “atypical” variant, in which HT is not accompanied by hypokalaemia; and a third “non-functioning” variant, which occurs without HT and with normal levels of potassium10,11.
Plasma renin activity (PRA) and plasma aldosterone (PA)
https://www.revistanefrologia.com/en-juxtaglomerular-cell-tumour-as-curable-articulo-X2013251415054971
SIGN
What sign is this and what does it indicate?
Case Discussion
Left sided duplicated collecting system with the upper moiety obstruction, and non-functioning. It displaces the lower pole moiety inferiorly, mimicking the appearance of a drooping lily.
The drooping lily sign refers to the inferolateral displacement of the opacified lower pole moiety due to an obstructed (and unopacified) upper pole moiety in duplicated collecting system.
Case courtesy of Dr Mohammad Taghi Niknejad, Radiopaedia.org, rID: 54354
What are the 6 radiographic features of uretal duplication?
What sign is this?
Radiographic Features (Lebowitz) ( Fig. 11.39 )
- Increased distance from top of nephrogram to collecting system: hydronephrotic upper pole moiety causes mass effect (1)
- Abnormal axis of collecting system (2)
- Concave upper border of renal pelvis (3)
- Diminished number of calyces compared with normal side; drooping lily sign (4)
- Lateral displacement of kidney and ureter (5)
- Spiral course of ureter (6)
- Filling defect in the bladder (ureterocele)
Case courtesy of Dr Mohammad Taghi Niknejad, Radiopaedia.org, rID: 54354
What is a renal Adenoma?
Adenoma
Best described as adenocarcinoma with no metastatic potential. Usually detected at autopsy.
Peripelvic Cyst
- Originates from sinus structures, most likely lymphatic in origin
- May be indistinguishable from hydronephrosis on US, requiring an IVP or CT for definitive diagnosis
- Attenuated, stretched infundibula
- IVP differential diagnosis (DDx): renal sinus lipomatosis
PATHOLOGICAL SPECIMEN
- What is the inheritance pattern?
- What is the incidence?
- What are the clinical findings?
- What is the treatment?
- What is the malignancy risk?
Adult Polycystic Kidney Disease (Apkd) ( Fig. 4.5 )
- Intro:
- Cystic dilatation of collecting tubules, as well as nephrons
- (unlike MCD and infantile polycystic kidney disease in which only the collecting tubules are involved).
- AD trait (childhood type is AR).
- Incidence:
- 0.1% (most common form of cystic kidney disease; accounts for 10% of patients on chronic dialysis).
- Clinical
- Slowly progressive renal failure.
- Symptoms usually begin in 3rd or 4th decade, but clinical onset is extremely variable, ranging from palpable cystic kidneys at birth to multiple cysts without symptoms in old age.
- Enlarged kidneys may be palpable.
- Treatment
- Treatment is with dialysis and transplant.
- Malignancy
- No increased risk of malignancy.
What are the complications of RF Ablation (re RCC)?
Complications
- Pain may last for several days to weeks.
- Postablation syndrome:
- fever, malaise, and body aches.
- Severity is related to volume of tissue ablated.
- Hemorrhage, often self-limited
- Injury to ureter, central collecting system, or adjacent organs
What are the 6 associations of this condition?
Horseshoe Kidney
- Associations
- UPJ obstruction, 30%
- Ureteral duplication, 10%
- Genital anomalies
- Other anomalies: anorectal, cardiovascular, musculoskeletal anomalies
Case courtesy of Dr Aditya Shetty, Radiopaedia.org. From the case rID: 27958
What is the staging for this condition?
Renal pelvis TCC
Staging
- Stage I: mucosal lamina propria involved
- Stage II: into, but not beyond, muscular layer
- Stage III: invasion of adjacent fat/renal parenchyma
- Stage IV: metastases
Medullary Cystic Disease
What is the cause of this disease?
Medullary Cystic Disease (MCD) ( Fig. 4.4 )
Spectrum of diseases characterized by tubulointerstitial fibrosis.
Patients usually present with azotemia and anemia and subsequently progress to end-stage failure.
Azotemia: Elevated levels of urea and other nitrogen compounds in the blood.
SYNDROMES
What syndromes/diseases are a/w renal Cysts?
What are the differences in the morphology of the cysts?
- TS
- VHL
- BHD
- ARPCKD
- ADRCKD
- DICER
- Nephronopthisis
- Asphyxiating thoracic dys- trophy (Jeune syndrome)
- Meckel-Gruber syndrome;
- Joubert syndrome and related disorders;
- Ellis–van Creveld syndrome;
- short rib–polydactyly group
Fig. 63.1 (A) Postcontrast CT image shows bilateral renal cysts. There is a large cyst within the right kidney with thick enhancing septations (arrowhead). Note the multiple pancreatic cysts (arrow). (B) Axial image shows a mixed solid and cystic lesion in the left kidney (arrowhead), along with an enhancing nodule in the chord (arrow). (C)An additional complex cyst with enhancing septation is seen in the right kidney (arrowhead), along with a small enhancing lesion in the left kidney (arrowhead).
SYNDROMES
Re ADPCKD describe the following:
Type of inheritance?
Genes?
Renal findings?
Associated findings?
AD
PKD1, PKD2
(ciliopathy)
Multiple bilateral variably sized renal cortical and medul-
lary cysts; number and size
of cysts increase with time; nephrolithiasis; chronic kidney disease typically occurs in late adulthood
Ductal plate malformations (eg, congenital hepatic fibrosis); extrarenal cysts (eg, liver, pancreas, seminal vesicles); intracranial aneu- rysms (typically in adult- hood)
Describe the staging system of RCC
Staging
- Stage I:
- tumor confined to kidney
- Stage II:
- extrarenal (may involve adrenal gland) but confined to Gerota fascia
- Stage III:
- A: venous invasion (renal vein);
- B: lymph node metastases;
- C: both
- Stage IV:
- A: direct extension into adjacent organs through Gerota fascia;
- B: metastases
- Lungs, 55%
- Liver, 25%
- Bone, 20% (classic lesions are lytic, expansile)
- Adrenal, 20%
- Contralateral kidney, 10%
- Other organs, <5%
Complicated Cysts
- Complicated cysts are cysts that do not meet the criteria of simple cysts and thus require further workup.
- Case Discussion
- This is a classic example of a Bosniak type IV cyst - with an abnormal cyst first identified on ultrasound proceeding to three phase CT imaging.
- It is made more difficult by the bilateral cystic renal disease, but the left mid/upper pole cyst contained a significant solid element.
- The patient is due for a total nephrectomy - the histopathological outcome will be added when available.
Case courtesy of Dr Ian Bickle, Radiopaedia.org, rID: 24244
KIDNEY ANATOMY
L and R kidneys should be within x cm of eachother?
Left and right kidney size should not vary more than 1cm.
What is the prognosis of RCC?
5 yr survival
Stage 1, 2, 3, 4?
What are the unual behaviours of RCC?
Prognosis
5-year survival:
- stage I = 81%
- stage II = 74%
- stage III = 53%
- stage IV = 8%
- Tumors often have atypical behavior:
- Late recurrence of metastases:
- 10% recur 10 years after nephrectomy.
- Some patients survive for years with untreated tumor.
- Spontaneous regression of tumor has been reported but is very rare.
- Late recurrence of metastases:
Dromedary hump: parenchymal prominence in left kidney; results from compression of adjacent spleen
Case courtesy of Assoc Prof Craig Hacking, Radiopaedia.org, rID: 53697
Angiomyolipoma (Aml)
- Hamartomas containing fat, smooth muscle, and blood vessels.
- Small lesions are not treated; large and symptomatic lesions are resected or embolized.
- Unlikely to bleed if <4 cm.
- Complication: tumors may spontaneously bleed because of their vascular elements.
Case courtesy of Dr Behrang Amini, Radiopaedia.org, rID: 35925
Axial CT through the kidneys shows a fat-attenuation lesion in the left kidney with a perirenal collection, most likely blood. This is consistent with a bleeding angiomyolipoma.
What are the 4 types of this condition?
Medullary cystic disease
Types
- Familial nephronophthisis, 70%, autosomal recessive (AR)
- Juvenile type, onset at age 3–5 years (most common)
- Adult MCD, 15%, autosomal dominant (AD)
- Renal-retinal dysplasia, 15%; recessive associated with retinitis pigmentosa
Radiopedia
- Medullary cystic disease complex belongs to group of paediatric cystic renal diseases characterised by progressive tubular atrophy with glomerulosclerosis (chronic tubulointerstitial nephritis) and multiple small medullary cysts.
- Epidemiology
- There is no recognised gender predilection
- Clinical presentation
- Presentation with polydipsia and polyuria, due to initial tubular injury, tends to progress to end stage renal failure, growth retardation, lethargy.
- Three clinical variants based on age of onset for end stage renal disease (ESRD):
- infantile: before 2 years of age
- juvenile (a.k.a. nephronophthisis): most common form, age of onset 10
- adolescent (a.k.a. medullary cystic kidney disease): usually develops in patients in their thirties
- There can be a clinical triad comprising of uraemia, anaemia, and salt wasting (hyponatraemia, hypokalaemia).
- Pathology
- It comprises a group of related conditions characterised by multiple cysts typically at the corticomedullary junction and medulla. The medullary cysts are small. There can be associated atrophy and fibrosis of the basement membrane of the proximal and distal tubules which leads into interstitial fibrosis and end stage renal disease.
- Variants
- familial nephronophthisis:
- autosomal recessive (40%)
- sporadic: non-familial (20%)
- retinal renal syndrome:
- autosomal recessive (15%) associated with retinitis pigmentosa
- adult onset medullary cystic disease:
- autosomal dominant (15%)
- familial nephronophthisis:
- Radiographic features
- Normal to small kidneys with multiple small (<1.5 cm) medullary cysts (sometimes cysts are too small to visualise) at the corticomedullary junction.
What is RF Ablation
Re RCC?
What are the 5 indications?
Radiofrequency Ablation of RCC
- Radiofrequency, cryoablation, and microwave ablation are noninvasive options for treating RCC.
- High-frequency alternating current is applied to a metallic applicator placed within the tumor.
- The surface area of the electrode is small, which results in a high current density at the electrode surface resulting in heat.
- When tissues reach temperatures greater than 50°C, they undergo necrosis.
Indications
- Comorbidities and contraindications to conventional or laparoscopic surgery
- Refusal of conventional surgery
- Compromised renal function
- Patients who have undergone a nephrectomy
- High risk of recurrence (e.g., VHL disease)
What are the imaging features of AML?
Imaging Features
- Fat appears hypodense (CT), hyperechoic (US), and hyperintense (T1W).
- The presence of fat in a renal lesion is virtually diagnostic of AML. There have been only a few case reports of fat in RCC or in oncocytomas.
- Five percent do not demonstrate fat on CT.
- Caveat: be certain that fat associated with a large mass is not trapped in renal sinus or peripheral fat.
- Predominance of blood vessels
- Strong contrast enhancement
- T2W hyperintensity
- Predominance of muscle:
- signal intensity (SI) similar to that of RCC
- AMLs do not contain calcifications;
- if a lesion does contain calcification, consider other diagnosis, such as RCC.
- Angiography:
- tortuous, irregular, aneurysmally dilated vessels are seen in 3%.
- Presence depends on the amount of angiomatous tissue.
- Predominantly myxomatous AMLs may be hypovascular.
Case Discussion
- Predominantly echogenic lesion involving the upper pole of the right kidney associated with more complex appearing perinephric collection.
- CT scan of the abdomen confirms the presence of a fatty lesion in the upper pole of the right kidney consistent with angiomyolipoma complicated by haemorrhage.
- Incidental two tiny hepatic haemangiomas.
- Perirenal haemorrhage without a history of trauma complicating an angiomyolipoma is known as Wunderlich syndrome.
- If perirenal haemorrhage is encountered on ultrasound, a thorough workup should be performed to exclude a bleeding renal cell carcinoma.
- Case courtesy of Dr Hani Makky Al Salam, Radiopaedia.org, rID: 10090
what is this condition
who does it happen to?
- Cystic nephromas, previously known as multilocular cystic nephromas, are rare benign renal neoplasms classically occurring in adult females in the 4th and 5th decades.
- As of the 2016 WHO classification, they are considered distinct from paediatric cystic nephromas which have associated DICER1 gene mutation 10,11,14.
- All different disorders:
- cystic nephroma (adult)
- paediatric cystic nephroma (paediatric)
- cystic partially differentiated nephroblastoma (paediatric)
- As of 2016, the WHO classifies cystic nephroma within the mixed epithelial and stromal tumour (MEST) family along a spectrum of disease 10,13,14. The remainder of this article will discuss the adult form of cystic nephroma.
-
Epidemiology
- overall rare
- adult (typically in the 5th - 6th decades)
- females are predominantly affected
Case courtesy of Dr Roberto Schubert, Radiopaedia.org, rID: 17489
What are are the risk factors for RCC?
What is a/w bilateral tumours?
Risk Factors
- Tobacco use
- Long-term phenacetin use
- VHL disease (bilateral tumors)
- Chronic dialysis (>3 years)
- Family history
What is the management of this lesion?
Oncocytoma
These tumors arise from oncocytes (epithelial cell) of the proximal tubule. Although the majority of lesions are well differentiated and benign, these tumors are usually resected because of their malignant potential and the difficulty in differentiating them from RCC preoperatively.
Can consider performing biopsy followed by observation in poor surgical candidates.
Represent 5% of renal tumors.
Case courtesy of Dr Paul Simkin, Radiopaedia.org, rID: 32824
Milk of Calcium Cyst
- Not a true cyst but a calyceal diverticulum, which may be communicating or closed off
- Contains layering calcific granules (calcium carbonate)
- No pathologic consequence
- Case courtesy of Dr Mohammad Taghi Niknejad, Radiopaedia.org, rID: 62623
- Case Discussion
- A 17mm non-enhancing cyst like lesion is noted adjacent to middle calyces of left kidney; accompanied with dependent hyperdense material.
- Features on CT scan are compatible with left renal milk of calcium cyst. Considered an incidental benign finding that does not require further treatment unless symptomatic.
-
Radiopaedia
- Renal milk of calcium cysts refer to the appearance of a calcium precipitate found either within a calyceal diverticulum, that has lost communication with the collecting system, or within a simple renal cyst.
- Clinical presentation
- Renal milk of calcium cysts are typically asymptomatic.
- Radiographic features
- Plain radiograph
- on erect or decubitus films, renal milk of calcium cysts have a characteristic crescentic opacity with a sharp upper border
- will change shape to a more oval-shaped opacity on supine projections
- Ultrasound
- dependent echogenic material within a hypoechoic cyst that demonstrates reverberation artifact
- posterior shadowing is present when large volumes of milk of calcium are present
- more common in the upper pole of the kidneys
- Plain radiograph
- Treatment and prognosis
- Considered an incidental benign finding that does not require further treatment unless symptomatic.
- Differential diagnosis
- On supine plain radiographs, a renal milk of calcium cyst may be mistaken for a renal calculus.
- Milk of calcium can also occur within the gallbladder.
What are the clincal findings of RCC?
- Hematuria, 50%
- Flank pain, 40%
- Palpable mass, 35%
- Weight loss, 25%
Perinephric Cyst
- Located beneath the renal capsule
- Not true cysts; represent extravasated urine trapped beneath renal capsule (pseudocysts, uriniferous cysts, urinoma)
- CT scan of abdomen shows multiple perinephric cystic fluid collections compressing both the kidneys.
- https://www.researchgate.net/figure/CT-scan-of-abdomen-shows-multiple-perinephric-cystic-fluid-collections-compressing-both_fig2_275123460
What are the imaging features of Medullary Cystic Disease?
Imaging Features
- Small kidneys (as opposed to large kidneys in polycystic disease)
- Multiple small (<2 cm) cysts in medulla
- Cysts may be too small to resolve by imaging, but their multiplicity will result in increased medullary echogenicity and apparent widening of central sinus echoes.
- Cortex is thin and does not contain cysts.
- No Calcifications
KIDNEY ANATOMY
How long and wide are the kidneys usually?
Do IVPs over or under estimate renal length?
Orientation and Size of Kidneys
Kidneys are 3 to 4 lumbar vertebral bodies in length, 12–14 cm long and 5–7 cm wide.
Intravenous pyelograms (IVPs) overestimate the true renal length because of magnification and renal engorgement from osmotic diuresis.
List the 19 different types of renal tumours
4 categories
6,3,8,2
- Renal parenchymal tumors
- Renal cell adenocarcinoma, 80%
- Wilms tumor, 5%
- Adenoma (thought to represent early RCC)
- Oncocytoma
- Nephroblastomatosis
- Mesoblastic nephroma
- Mesenchymal tumors
- Angiomyolipoma (AML)
- Malignant fibrous histiocytoma (MFH)
- Hemangioma
- Other rare tumors
- Renal pelvis tumors
- Transitional cell carcinoma (TCC) <10%
- Squamous cell carcinoma
- Undifferentiated adenocarcinoma tumors
- Benign tumors:
- papilloma
- angioma,
- fibroma
- myoma
- polyp
- Secondary tumors
- Metastases
- Lymphoma
What are the imaging findings of RCC?
What are the contrast enhancement characteristics?
Calcs?
Cystic?
Filling defects?
Size?
What is angiography useful for?
Imaging findings
- Mass lesion: renal contour abnormality, calyceal displacement
- Large variability in signal characteristics on noncontrast CT and MRI depending on the degree of hemorrhage and necrosis
- Contrast enhancement is usually heterogeneous; strong contrast enhancement (>15 HU)
- Calcification, 10%
- Cystic areas (2%–5% are predominantly cystic)
- Filling defects (clots, tumor thrombus) in collecting system and renal veins.
- US appearance
- Hyperechoic: 70% of tumors >3 cm, 30% of tumors <3 cm
- Hypoechoic
- Angiography
- 95% of tumors are hypervascular.
- Caliber irregularities of tumor vessels are typical (encasement).
- Prominent AV shunting, venous lakes
- Angiography may be useful for detection in complicated and equivocal cases:
- Small tumors
- Underlying abnormal renal parenchyma
- VHL disease
- Preoperative embolization
What are the typical imaging features of Oncocytomas?
Imaging Features
- Central stellate scar (CT) and spoke wheel appearance (angiography) are typical but not specific (also seen in adenocarcinoma).
- Well-defined, sharp borders
- Radiographically impossible to differentiate from RCC
- Case Discussion
- A nephrectomy was undertaken for this large renal mass.
- It was a renal oncocytoma on the surgical pathology specimen.
- This typically has a central stellate scar, however the appearances are very similar to the central necrosis observed in many renal cell carcinomas.
- Case courtesy of Dr Ian Bickle, Radiopaedia.org, rID: 78952
What is the incidence of renal involvement in lymphoma?
Which type of lymphoma is more common?
Lymphoma Incidence of renal involvement in lymphoma is 5%
non-Hodgkin lymphoma [NHL] > Hodgkin disease at diagnosis and 30% at autopsy.
What are the imaging features of this condition?
Horseshoe Kidney
- The two kidneys are connected across the midline by an isthmus.
- This is the most common fusion anomaly (other fusion anomalies: cross-fused ectopia, pancake kidney).
- The isthmus may contain parenchymal tissue with its own blood supply or consist of fibrous tissue.
-
Imaging Features
- Abnormal axis of each kidney with lower calyx more medial than upper calyx
- Bilateral malrotation of renal pelvises in anterior position
- Isthmus lies anterior to aorta and inferior vena cava (IVC) but behind inferior mesenteric artery (IMA)
Case courtesy of Dr Aditya Shetty, Radiopaedia.org. From the case rID: 27958
CT PROTOCOL
what is the Stone Protocol
Stone Protocol
- Noncontrast CT of the abdomen and pelvis using 5 mm slice thickness
- Coronal and sagittal reconstructions with 3 mm slice thickness
CT KUB
Last revised by Dr Vikas Shah◉ on 06 Jul 2021
- Computed tomography of kidneys, ureters and bladder (CT KUB) is a quick non-invasive technique for diagnosis of urolithiasis. It is usually considered the initial imaging modality for suspected urolithiasis in an emergency setting 1.
- Indications
- suspected urolithiasis
- haematuria (typically in conjunction with a CT-IVU)
- flank pain
- Purpose
- The purpose of CT KUB is primarily to assess for the presence of urolithiasis. Location of the stone, size, and secondary signs of renal tract obstruction can then be used to gauge the likelihood of passage and guide further management 3.
Case courtesy of Dr Ian Bickle, Radiopaedia.org, rID: 84479
PROCEEDURE
What are the inidcations of Percutaneous Nephrostomy?
What are the steps?
Percutaneous Nephrostomy (Pcn)
- Refers to percutaneous drainage of renal collecting system by catheter placement. Complications: 2%–4% (extravasation of contrast, bleeding)
- Indications
- Hydronephrosis (acute, subacute obstruction)
- Pyonephrosis
Technique ( Fig. 4.2 )
- Preprocedure workup: Check coagulation status.
Cefazolin 2 g IV < 120 kg; 3 g IV > 120 kg and gentamicin 120 mg IV × 1
Penicillin-allergic patients: vancomycin as above and aztreonam 2 g IV
Review all radiographs and determine on plain film where kidneys are located, especially in relation to colon, spleen, and pleural reflections.
- Local lidocaine. Under US guidance, place a 20-gauge introducer system into a posterior, lower pole calyx.
- The inner needle stylet can be removed and free flow of urine checked. Aspirate a small amount for cultures and to mildly decompress the system when urine is obtained.
- Attach connector tubing and inject a small amount of contrast to confirm positioning at the tip of a calyx. Detach the connector tubing carefully to ensure that the needle stays in place.
- Use the microwire to gain access to the renal pelvis. Remove the introducer needle, and insert the plastic sheath that comes with the set.
- Place a 0.038 Amplatz wire through the sheath into the renal collecting system and down the ureter. Depending on the anatomy/obstruction, it may be necessary to use different wire combinations.
- Dilate skin up to 12 Fr.
- Pass 8–10-Fr percutaneous nephrostomy (PCN) catheter over guidewire. Remove stiffener and guidewire. Coil pigtail. Inject contrast to check position of catheter within the renal pelvis. Cut thread.
- In the setting of infection, complete evaluation of the ureter via an antegrade injection is best deferred to a second visit.
PATHOLOGY SPECIMEN
What are the associated findings of this condition?
Associated Findings
- Hepatic cysts, 70%
- Intracranial berry aneurysm, 20%
- Cysts in pancreas and spleen, <5%
What is the paraneoplastic syndrome a/w RCC and the associated hormones?
Paraneoplastic syndrome:
- HTN (renin),
- erythrocytosis (erythropoietin),
- hypercalcemia (PTH),
- gynecomastia (gonadotropin),
- Cushing syndrome (ACTH)
What are the imaging features of Renal Lymphoma?
Imaging Features
- Multiple lymphomatous masses (hypoechoic, hypodense), 50%
- Diffuse involvement of one or both kidneys
- Adenopathy
Case courtesy of Dr Abdallah Al Khateeb, Radiopaedia.org, rID: 47824