A case of fibrolamellar hepatocellular carcinoma (FL-HCC) associated with adjacent focal nodular hyperplasia (FNH) is described. These two regions were adjacent but distinct, both on gross and microscopic examination. Currently, it is unclear whether FL-HCC rarely arises in preexisting FNH, or whether FNH is a typical response to this vascular variant of hepatocellular carcinoma (HCC). The FNH region, which is peripheral, may be biopsied to exclude the underlying carcinoma, and thus lead to inadequate therapy. Previous reports of this association are reviewed. importance of this observation in terms of patient management and proposed pathogenesis is discussed.
Case ReportA 19-year-old woman with a 3-year history of oral contraceptive use (type unknown) was admitted for liver mass evaluation. One and one half months before she was admitted, sharp, episodic, left-sided chest pain developed. Hepatomegaly without splenomegaly was detected at evaluation. A computed tomography (CT) scan showed a hepatic mass. She was transferred to the Hospital of the University of Pennsylvania for further examination. The results of the physical examination were normal except for an 1 I-cm liver (by percussion), which was nontender but palpable 5.0 cm below the right costal margin. Complete blood count and serum electrolytes were normal, as were the alanine and aspartate aminotransferases and gamma glutamyl transpeptidase. Serum alkaline phosphatase was minimally elevated at 256 IU/L (compressed normal hepatic parenchyma < 140 IU/L). Serum alpha-fetoprotein and carcinoembryonic antigen were less than 10 ng/ml and 3 ng/ml, respectively. A nonenhanced and contrast-enhanced abdominal CT scan showed a large, relatively hypodense mass in the right hepatic lobe, with an enhancing central septate region. Selective right hepatic arteriography demonstrated a large hypervascular mass in the inferior portion of the right lobe, with a peripheral arterial supply radially penetrating into the center of the lesion (Figure 1). The parenchymal phase showed a dense capillary blush with a stellate septate pattern and central lucency compatible with a central scar. Vascular encasement. portal vein thrombosis, or arteriovenous shunting were not detected. The lesion was considered consistent with FNH.The patient was operated on and the anterior segment of the right hepatic lobe was removed and sent for pathologic con-
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The midaortic syndrome is an unusual entity seen in children and adolescents. It is characterized by severe narrowing of the abdominal aorta with progressive involvement of the renal and visceral branches. Eleven patients (aged 5 months to 15 years) suspected of having midaortic syndrome were examined preoperatively and postoperatively. All patients had hypertension and were examined with midstream aortography. All aortograms showed a smooth, segmental stenosis of the abdominal aorta and severe bilateral proximal renal artery stenosis. In three patients, percutaneous transluminal angioplasty of the renal artery was attempted, two preoperatively and one for a postoperative stricture. None showed long-term success, presumably due to the progressive nature of the disease. Grafts were surgically placed in ten patients and produced successful results in nine.
Radiologically guided balloon catheters were used to dilate 94 gastrointestinal strictures in 92 patients over a 6-year period. Fifty strictures were esophageal and 44 nonesophageal (22 gastroenterostomies, 11 antral-pyloric strictures, four colorectal strictures, four enteroenterostomies, and three miscellaneous strictures). Factors influencing the success of stricture intubation included patient age, stricture location (esophageal vs. nonesophageal and proximal vs. distal esophageal), and association with a surgical anastomosis. Malignancy was associated with greater postdilation irregularity and a smaller increase in stricture diameter, as measured radiographically. Procedural failures occurred in 8% of cases (2% of esophageal and 30% of nonesophageal lesions). Two small, asymptomatic mucosal tears were seen after dilation (one esophageal and one colonic); no other procedural complications occurred. Following successful dilation, 16 patients (17%; six with esophageal and ten with non-esophageal strictures) had recurrence of symptoms during short-term (30-day) follow-up.
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