Cases reported "Focal Nodular Hyperplasia"

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1/6. Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin-Johnson syndrome.

    We report a rare case of congenital extrahepatic portocaval shunt diagnosed during evaluation of hyperplastic nodules in the liver. Diagnostic imagings showed hypoplasia of the intrahepatic portal venous system and splanchnic portal venous return to the inferior vena cava through aberrant vessels. Altered hepatic blood flow dynamics due to this shunt may have been implicated in the etiology of the hepatic hyperplastic nodules.
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2/6. Hepatic nodular hyperplasia in a boy with alagille syndrome: CT and MR appearances.

    A boy with alagille syndrome complicated by hepatic nodular hyperplasia (HNH) is reported. During pre-transplantation evaluation, CT and MRI revealed a large hepatic lesion with multiple small nodular lesions. angiography demonstrated a large hypervascular lesion and CT arterial portography (CTAP) showed a portal perfusion defect corresponding to the lesion. CTAP also revealed large portal vein branches running through the lesion. Although hepatocellular carcinoma is known to accompany alagille syndrome, HNH should also be considered when large vessels running through the lesion are demonstrated.
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3/6. Large focal nodular hyperplasia of the liver: possible to evade surgical resection.

    PURPOSE: Although recent advances in diagnostic imaging have allowed a number of patients with focal nodular hyperplasia (FNH) to avoid surgical treatment, the natural course of large FNH is still unknown. CASE REPORT: A 25-year-old man was admitted because of a large hepatic mass detected on routine examination in June 1998. The only laboratory abnormality was an elevated gamma-GTP. Computed tomography, angiography, positron emission tomography using F-18 fluorodeoxyglucose (FDG-PET), and scintigraphy using technetium-99m-galactosylneoglycoalbumin(99mTc-NGA) demonstrated a spoke-wheel appearance of vessels, normal hepatocytes, and no malignancy. Histologic findings on needle biopsy were consistent with FNH. After informed consent, the patient agreed to observation. Two years after the initial diagnosis, he has no symptoms, and there are no changes in the size or character of the lesion on computed tomography. DISCUSSION: Careful observation of patients with FNH is required because its natural course is unknown and these lesions can bleed or rupture.
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4/6. Spindle cell fragments in focal nodular hyperplasia of the liver. A case report.

    BACKGROUND: There are only few reports on the fine needle aspiration cytology (FNAC) findings of focal nodular hyperplasia (FNH) of the liver. CASE: A 30-year-old woman who had undergone surgery for a leiomyosarcoma of the calf, was found to have a hepatic mass five years later on imaging during routine follow-up. Fine needle aspiration was performed to rule out metastasis. Cytology revealed a few fragments of bland-looking spindle cells in a metachromatic stroma along with benign hepatocytes and bile duct cells. It was interpreted as "consistent with metastasis of leiomyosarcoma." The excised mass showed histologic features typical of FNH. CONCLUSION: Spindle cell fragments have not been previously observed in the FNAC of FNH. These fragments probably represent the muscular wall of the abnormal blood vessels of FNH. If smooth muscle fragment is seen accompanying benign hepatocytes and bile duct cells, one should consider the diagnosis of FNH in the needle aspirate.
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5/6. Telangiectatic focal nodular hyperplasia of the liver: a case detected at birth.

    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.
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6/6. Atypical laparoscopic resection of a liver tumor in a 4-year-old girl.

    Despite the advances in minimally invasive surgery in children, considerable concern exists about employing such techniques in oncologic cases. We report our experience with a 4-year-old girl with a symptomatic tumor in the liver. Contrast-enhanced computed tomography revealed a 3 x 4 cm lesion, confined to segments II and III. Tumor markers were negative and true-cut needle biopsy did not rule out malignancy. We performed a diagnostic laparoscopy using four 5-mm ports. Since the tumor did not cause any alterations of the liver surface, a 5-mm flexible endoscopic ultrasound probe (5 MHz) was applied to reveal the extent of the tumor. Parenchymal dissection was performed with a radiofrequency probe, and the LigaSure device was used to seal larger vessels and bile ducts. The tumor was resected completely and removed in a specimen bag via the umbilical incision. histology revealed fibrous nodular hyperplasia. The postoperative course was uneventful and the girl was discharged on postoperative day 5. We conclude that laparoscopic resection of confined liver lesions is feasible in children, employing standard principles of oncologic surgery and safety.
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