We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities.
Adult ; Female ; Focal Nodular Hyperplasia/*radiography ; Humans ; Hypertension, Portal/*complications ; Hypertension, Pulmonary/*complications ; Portal Vein/*abnormalities

Focal nodular hyperplasia (FNH) is the second most common benign solid tumor of the liver and is usually found in young females. In FNH, spontaneous bleeding or rupture rarely occurs and malignant transformation is unlikely. The etiology of FNH is unclear, but because of female predominance and young age at onset, it seems that female hormone has an important role for the development of FNH. Although the development and the complications of hepatocellular adenomas have been related to the use of oral contraceptives and pregnancy, the influence of oral contraceptives and pregnancy on the growth and complications of FNH is controversial. Most FNH are stable in size and rarely complicated during pregnancy. We describe here a case of FNH with growth progression during pregnancy in a 27-year-old female. Her course of pregnancy and delivery was uneventful. Two months after delivery, the size of FNH was decreased.
Adult ; Female ; Focal Nodular Hyperplasia/*diagnosis/radiography/ultrasonography ; Humans ; Liver/pathology/radiography ; Pregnancy ; Tomography, X-Ray Computed ; alpha-Fetoproteins/analysis

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

BACKGROUND/AIMS: Focal nodular hyperplasia (FNH) is mandatory to be differentiated from other hepatic tumorous conditions such as hepatocellular carcinoma and adenoma. The purpose of this study was to explore the clinical, radiological and pathological features of FNH cases reported in Korea. METHODS: We have searched the journals from the web site "http://koreamed.org" using keywords "focal nodular hyperplasia" and "liver" - total of 38 cases of FNH, 37 cases from 17 published articles and one case from our experience confirmed histologically, were reviewed and analyzed. RESULTS: Thirty eight cases were diagnosed between gestational age of 36 weeks and 67 years. Seventeen female patients (45%) had no history of taking oral contraceptives. Twenty cases (52.6%) experienced clinical symptoms such as abdominal pain and palpable mass. Computed tomography revealed contrast-enhancement in 34 nodules (85%) and typical central stellate scar in 9 (22.5%) of 40 nodules. Magnetic resonance imaging showed T1 weighted low signal in 18 (60%) and T2 weighted high signal in 22 (73.3%) of 30 nodules. Six (60%) of 10 cases showed hypervascular staining on hepatic angiography. Among 38 cases, 32 (84.2%) cases had single nodule and their mean size was 3.9 cm (0.5-16 cm). Pathologically, fibrous septa, proliferation of bile ductules and arterial wall thickening were seen in most cases. CONCLUSIONS: Of all the FNH cases reported in Korea, there were some differences in clinical aspects of sex ratio, accompanying clinical symptoms, and relationship with oral contraceptives, compared with previous reports. Further prospective studies are needed by means of nation-wide clinical survey and analysis.
Adolescent ; Adult ; Aged ; Child ; Contraceptives, Oral ; Female ; Focal Nodular Hyperplasia/*diagnosis/pathology/radiography ; Humans ; Korea ; Male ; Middle Aged ; Sex Factors ; Tomography, X-Ray Computed