VHL syndrome
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Findings
Simple cyst is seen in segment V/VII of liver.
Multiple arterial phase enhancing masses are seen in both kidneys. Few of them show homogenous enhancement and few show heterogenous enhancement with large necrotic component. Largest mass is seen in in lower pole of right kidney, measures 8.7x6.9cm . Arterio venous shunting is seen in this mass. Bilateral perinephric fat stranding is seen.
An enhancing mass measuring 2.1x1.9cm is seen in left renal pelvis. Mild left hydro-uretero-nephrosis is seen.
Hypodense filling defect is seen in mid part of left renal vein – likely due to thrombosis. Few venous collaterals seen in retroperitonium. Right renal vein and IVC show dilatation with no obvious filling defect within.
Few simple cysts are seen in both kidneys.
Arterial phase enhancing lesion measuring 5x3.1cm is seen in right adrenal gland – likely pheochromocytoma. HU value on plain 36, HU value on contrast 54. Scalloping of segment VII of liver is seen.
Lytic metastatic lesion is seen in L4 vertebral body. Large lytic expansile metastatic lesion with associated soft tissue component is seen in sacrum encasing sacral nerve roots.
Multiple arterial phase enhancing masses are seen in both kidneys. Few of them show homogenous enhancement and few show heterogenous enhancement with large necrotic component. Largest mass is seen in in lower pole of right kidney, measures 8.7x6.9cm . Arterio venous shunting is seen in this mass. Bilateral perinephric fat stranding is seen.
An enhancing mass measuring 2.1x1.9cm is seen in left renal pelvis. Mild left hydro-uretero-nephrosis is seen.
Hypodense filling defect is seen in mid part of left renal vein – likely due to thrombosis. Few venous collaterals seen in retroperitonium. Right renal vein and IVC show dilatation with no obvious filling defect within.
Few simple cysts are seen in both kidneys.
Arterial phase enhancing lesion measuring 5x3.1cm is seen in right adrenal gland – likely pheochromocytoma. HU value on plain 36, HU value on contrast 54. Scalloping of segment VII of liver is seen.
Lytic metastatic lesion is seen in L4 vertebral body. Large lytic expansile metastatic lesion with associated soft tissue component is seen in sacrum encasing sacral nerve roots.
Diagnosis
Arterial phase enhancing masses in both kidney and left renal pelvis – suggestive of renal cell carcinomas.
Hepatic and bilateral renal simple cysts.
Ø Right adrenal arterial phase enhancing mass - likely pheochromocytoma.
Ø Skeletal metastatic deposits.
Ø Left renal vein thrombosis.
Ø Overall findings likely represents VHL syndrome- clinical sos further evauation with MR imaging of brain/ orbit / spine is suggested.
Hepatic and bilateral renal simple cysts.
Ø Right adrenal arterial phase enhancing mass - likely pheochromocytoma.
Ø Skeletal metastatic deposits.
Ø Left renal vein thrombosis.
Ø Overall findings likely represents VHL syndrome- clinical sos further evauation with MR imaging of brain/ orbit / spine is suggested.
Dr Rupali Hanmant Kshirsagar MD is a Consultant Radiologist from with experience of 1-2 year post-graduated from Tata Memorial Centre , Mumbai. Specialized in Reporting of X-RAYS , CT-SCAN , MRI , MAMMOGRAM cases with expertise in Neuro-imaging , Spine-imaging , Stroke-imaging , Breast-imaging
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