No abstract available.
Hepatic Encephalopathy/*congenital ; Humans ; Liver Neoplasms/*congenital ; Male ; Portal Vein/*abnormalities ; *Septal Occluder Device

A case of telangiectatic focal nodular hyperplasia (FNH) was detected at birth and was surgically removed. Grossly, the lesion was a solitary nodule and showed vague nodularity, appearing as an adenoma-like mass with fine fibrous septa, but having no macroscopic scar. On microscopic scale, the mass typically had neither fibrous central scar nor hyperplastic nodules different from the usual FNHs. The hepatic plates were separated by sinusoidal dilatation, sometimes alternating with areas of marked ectasia. Instead of large fibrous scar, thin fibrous septa were often found, and contained abnormal tortuous large arteries. These high-pressure vessels were connected directly into the adjacent sinusoids and made marked dilation of sinusoids. Bile ductular proliferation was also noted in the thin fibrous septa. To our knowledge, this is considered to be the first reported case of telangiectatic FNH detected at birth.
Focal Nodular Hyperplasia/*congenital/*diagnosis/radiography ; Human ; Hyperplasia ; Infant ; Liver/pathology ; Liver Neoplasms/congenital/*diagnosis/radiography ; Male ; Tomography, X-Ray Computed

Congenital mesoblastic nephroma (CMN) is the most common renal tumor in the first year of life. Here, we present unique findings of cellular variant CMN seen on prenatal and postnatal MRI with diffusion-weighted imaging (DWI).The mass was well-visualized on prenatal MR DWI with diffusion restriction in the solid portions. After excision of the mass, follow-up whole body MRI with DWI helped identify local tumor recurrence with suspicious liver metastasis. This hepatic lesion also showed diffusion restriction.
Adult ; Combined Modality Therapy ; Diffusion Magnetic Resonance Imaging/*methods ; Female ; Humans ; Infant, Newborn ; Kidney Neoplasms/*congenital/therapy ; Liver Neoplasms/*secondary/therapy ; Nephroma, Mesoblastic/*congenital/therapy ; Pregnancy

A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been reported, and the mass is another reason for treatment. Authors report a case of a congenital intrahepatic portosystemic shunt with a hepatic mass that was successfully treated using a percutaneous endovascular approach with vascular plugs. By the time the first short-term follow-up was conducted, the hepatic mass had disappeared.
Child ; Diagnosis, Differential ; Hepatic Encephalopathy/*congenital/diagnosis/therapy ; Humans ; Liver Neoplasms/*congenital/diagnosis/therapy ; Male ; Portal Vein/*abnormalities ; *Septal Occluder Device ; Tomography, X-Ray Computed ; Ultrasonography, Doppler, Color ; Ultrasonography, Interventional

Diagnosis, Differential ; Fetus ; Fibroma ; metabolism ; pathology ; Fibrosarcoma ; congenital ; genetics ; metabolism ; secondary ; Hemangiopericytoma ; metabolism ; pathology ; Humans ; Kidney Neoplasms ; pathology ; secondary ; Liver Neoplasms ; pathology ; secondary ; Lung Neoplasms ; pathology ; secondary ; Male ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Rhabdomyosarcoma, Embryonal ; metabolism ; pathology ; Soft Tissue Neoplasms ; congenital ; genetics ; metabolism ; pathology ; Vimentin ; metabolism