Von Hippel-Lindau (VHL) syndrome is a rare neoplastic disorder characterized by central nervous system (CNS) and visceral tumors. We here present 68Ga-labelled [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-Nal3-Octreotide positron emission tomography computed tomography findings in a 52 year old female with VHL syndrome, demonstrating both CNS and visceral tumors.
Brain Diseases/radionuclide imaging ; Female ; Humans ; Kidney Diseases/radionuclide imaging ; Liver Diseases/radionuclide imaging ; Middle Aged ; Multimodal Imaging/*methods ; Organometallic Compounds/diagnostic use ; Pancreatic Diseases/radionuclide imaging ; Positron-Emission Tomography/*methods ; Tomography, X-Ray Computed/*methods ; von Hippel-Lindau Disease/*radionuclide imaging

PURPOSE: Heart to liver ratio on T1-201 per rectal scintigraphy (shunt index) is known to be useful in the assessment of portal systemic shunt. We assessed T1-201 uptake pattern and early liver/heart uptake rate of T1-201 and correlated with shunt index in patients with chronic active hepatitis (CAH) and liver cirrhosis (LC). MATERIALS AND METHODS: Fifty eight patients with biopsy-proven chronic liver disease (35 with CAH, 23 with LC) underwent T1-201 per rectum scintigraphy after instillation of 18.5 MBq of T1-201 into the upper rectum. We evaluated hepatic uptake (type 1: homogeneous, 2: inhomogeneous segmental, 3: inhomogeneous nonsegmental) and extrahepatic uptake of spleen, heart and kidney (grade 0: no uptake, 1: less than liver, 2: equal to liver, 3: greater than liver). We measured the early liver/heart uptake rate (the slope of the liver to heart uptake ratio for 10 min) and shunt index (heart to liver uptake ratio). T1-201 uptake pattern and early liver/heart uptake rate of T1-201 was correlated with the pathologic diagnosis and shunt index. RESULTS: Hepatic uptake patterns of type 1 and 2 were dominant in CAH (CAH: 27/35, LC: 8/23), and type 3 in LC (CAH: 8/35, LC: 15/23) (p<0.005). The grades of extrahepatic uptake were higher in LC than in CAH (spleen: p<0.001, other soft tissue: p<0.005). The early liver/heart uptake rate of CAH (0.110+/-0.111) was significantly higher than that of LC (0.014+/-0.090) (p<0.001). The sensitivity and specificity of the early liver/heart uptake rate were 77.7% and 67.7% in differentiating LC from CAH. There was negative correlation between early liver/heart uptake rate and shunt index (r=-0.3347, p<0.01). CONCLUSION: Hepatic and extrahepatic uptake pattern and early liver/heart uptake rate on T1-201 per rectum scintigraphy are useful in the assessment of portal systemic shunt in patients with chronic liver disease.
Diagnosis ; Heart ; Hepatitis, Chronic ; Humans ; Kidney ; Liver Cirrhosis ; Liver Diseases* ; Liver* ; Portal System ; Radionuclide Imaging* ; Rectum* ; Sensitivity and Specificity ; Spleen