Association between Klinefelter syndrome and focal nodular hyperplasia

Focal nodular hyperplasia is a benign lesion of the liver, predominantly affecting women. Its etiology is unknown. Elevated levels of estrogens have been invoked to play a role in the disease. Klinefelter syndrome is the most common sex chromosome disorder, characterized by 47, XXY karyotype, resulting in male hypogonadism and sex hormone imbalance. We present a case of a 25-year-old man affected by Klinefelter syndrome, admitted to our hospital for aspecific dyspeptic symptoms. During admission he underwent: blood test for the liver function and sexual hormonal status, ultrasonography, echo color power Doppler and computerized tomography scan of the liver, and liver biopsy. A hypergonadotropic hypogonadism was present. Imaging of the liver showed an hepatic lesion that liver biopsy confirmed to be a focal nodular hyperplasia. Although the association could be casual, the sex hormone imbalance present in Klinefelter syndrome may suggest a role in the development of this benign liver lesion.     

Management of spontaneous hepatic hemorrhage due to focal nodular hyperplasia

Focal nodular hyperplasia is a benign liver tumor that usually follows an asymptomatic course. Hemorrhage of hepatic focal nodular hyperplasia is exceptional and occurs in tumors such as hepatocellular carcinoma and hepatic adenoma. We report the case of a woman with spontaneous rupture and hemorrhage of focal nodular hyperplasia. Hemodynamic stabilization was achieved by selective hepatic arterial embolization. Elective hepatic resection was subsequently performed. This clinical course is extremely rare. We describe the therapeutic management of these complications using hepatic transarterial embolization, which could avoid potentially harmful aggressive treatments.     

Multiple focal nodular hyperplasia

Focal nodular hyperplasia (FNH) is a benign lesion of the liver which usually presents with one or two localizations. We report a patient with history of resection of a biliary cyst, and who had been taking oral contraceptives for the past 18 years, who had multiple localizations of FNH (more than 30 lesions). The largest lesion measured 10.5 × 11 × 12 cm. The imaging characteristics of our patient were atypical. A central scar could be demonstrated only in the largest lesion, in an eccentric location. In the other lesions, no scar formations could be detected. Futhermore, imaging characteristics suggested that several of the lesions contained fat. This was confirmed by biopsy. The patient had an associated inflammatory syndrome which could not be otherwise explained. The patient was advised to stop taking the oral contraceptives. Follow-up after 2 years showed that the lesions were unchanged; the inflammatory syndrome persisted. Multiple localizations of FNH are very rare. Sometimes they are associated with malformations in other organs (vascular malformations and neoplasia, mostly of the brain). Often they occur as isolated cases, however. Usualy their prognosis seems to be good. (Received Jan. 14, 1998; accepted May 22, 1998)     

Liver nodules resembling focal nodular hyperplasia after hepatic venous thrombosis

We report the case of focal nodular hyperplasia-like nodular hepatic lesions, that developed in the liver of a 35-year-old Caucasian female who required orthotopic liver transplantation for Budd-Chiari syndrome. The rapid development of focal nodular hyperplasia-like lesions in a severe hepatic vascular disorder and in the absence of cirrhosis may represent an additional argument in favor of the vascular origin of focal nodular hyperplasia. The pathogenesis of the nodules is not clear, but pathological arterialization of the liver in hepatic vein thrombosis may be a candidate mechanism.     

Spontaneous rupture and hemorrhage of hepatic focal nodular hyperplasia in lobus caudatus

In general, focal nodular hyperplasia lesions of the liver have a benign natural course; the majority of cases remain asymptomatic and complications are rare. We report a case of spontaneous rupture and hemorrhage of focal nodular hyperplasia, which is extremely rare in the literature. A 35-year-old woman was admitted with severe upper abdominal pain and unstable hemodynamic status. No major abdominal trauma was noted. Radiology findings suggested a diffuse hemoperitoneum. Emergent surgical exploration showed a hemoperitoneum due to the rupture of a hepatic mass lesion in segment I. Suture of the rupture was undertaken with success. After this lifesaving emergent surgery, further investigations, including ultrasound, contrast-enhanced computed tomography, and magnetic resonance imaging, were undertaken, and a 5.7 × 9.8 × 6.4-cm focal nodular hyperplasia lesion was identified in segment I. Two weeks after the first surgical hemostasis, surgical removal of segment I, including the mass, was performed. The postoperative course was uneventful. Pathological evaluation confirmed the nature of focal nodular hyperplasia. The patient remains asymptomatic without evidence of recurrence 3 years and 6 months after surgery. To our knowledge, this is the firstcase of spontaneous rupture and hemorrhage of focal nodular hyperplasia that needed two consecutive surgical operations.     

Contrast-enhanced, wide-band phase-inversion power Doppler imaging of hepatic focal nodular hyperplasia

BACKGROUND/AIMS: To determine whether examination of hepatic focal nodular hyperplasia by wide-band, phase-inversion sonography offers any advantages over power and color Doppler sonography in the depiction of specific characteristics of these pathologies. METHODOLOGY: Twenty-six patients were examined. The presence of focal nodular hyperplasia was confirmed by ultrasound-guided biopsy, surgical resection, dynamic helical computed tomography or magnetic resonance. All patients, prior to enhanced sonography, had undergone B-mode gray-scale sonography, color Doppler, and power Doppler examinations. After injection of 2.5 g of Levovist intravenously, analysis of the contrast agent arrival was performed by wide-band, phase-inversion power Doppler sonography. RESULTS: The B-mode gray-scale sonography, color and power Doppler sonography were non-specific for focal nodular hyperplasia in 14 cases in our examination. However based on the wide-band, phase-inversion power Doppler sonography findings all patients with focal nodular hyperplasia were diagnosed. All typical anatomic features of focal nodular hyperplasia such as "star sign" or "spoke-wheel" pattern were clearly visible. In 3 cases, computed tomography and magnetic resonance imaging had failed to disclose pathology while phase-inversion sonographic images were completely suggestive which was later finally confirmed by histologic examination. CONCLUSIONS: Our data demonstrate the usefulness of wide-band, phase-inversion power Doppler sonography in the differential diagnosis of hepatic focal nodular hyperplasia by visualizing all characteristic anatomic details.     

Diagnosis and treatment of focal nodular hyperplasia

Focal nodular hyperplasia is an uncommon, benign lesion of the liver of minimal clinical significance. Its importance lies in differentiating it from clinically significant lesions such as liver cell adenoma and hepatocellular carcinoma. The purpose of this review is to provide a background on the pathogenesis of focal nodular hyperplasia, discuss current imaging modalities which provide the most specificity in diagnosis, and provide an update on the accepted management of these lesions. While debate still exists, most current authors believe that focal nodular hyperplasia represents a local response of the liver to injury. The source of that injury seems to be vascular malformations. Magnetic resonance imaging (MRI) provides the most specificity in diagnosis, although the characteristics are still being described. Classically, the lesions will be isointense on T2-weighted images with rapid homogenous contrast enhancement and delayed enhancing central stellate scar. Surgical resection is limited to symptomatic lesions or lesions with atypical imaging characteristics suggesting the possibility of a malignant diagnosis. Percutaneous biopsy can be helpful in those lesions where there is an overall high suspicion of focal nodular hyperplasia, but some of the clinical aspects or imaging characteristics are atypical for the diagnosis. The use of embolization as first line therapy for symptomatic lesions deserves further investigation.     

Multiple hepatic focal nodular hyperplasia: its presentation in childhood and atypical evolution

Multiple focal nodular hyperplasia is an uncommon benign liver tumor although its incidence has been increasing in the last few years. A case of focal nodular hyperplasia in a young woman is described, which was discovered in infancy and which presented two nodules in each lobe. The diagnosis was subsequently confirmed by large surgical biopsy. Conservative therapy was given for 16 years during which time there was progressive tumor growth, increase of pain and cholestatic enzymes. The unusual presentation this benign lesion may have, a strategy for its diagnosis and the generally conservative management that is currently favored are discussed.     

Pictures of focal nodular hyperplasia and hepatocellular adenomas

This practical atlas aims to help liver and non liver pa-thologists to recognize benign hepatocellular nodules on resected specimen. Macroscopic and microscopic views together with immunohistochemical stains illustrate typical and atypical aspects of focal nodular hyperplasia and of hepatocellular adenoma, including hepatocel-lular adenomas subtypes with references to clinical and imaging data. Each step is important to make a correct diagnosis. The specimen including the nodule and the non-tumoral liver should be sliced, photographed and all different looking areas adequately sampled for par-affin inclusion. Routine histology includes HE, trichrome and cytokeratin 7. Immunohistochemistry includes glu-tamine synthase and according to the above results ad-ditional markers such as liver fatty acid binding protein, C reactive protein and beta catenin may be realized to differentiate focal nodular hyperplasia from hepatocel-lular adenoma subtypes. Clues for differential diagnosis and pitfalls are explained and illustrated.     

肝脏局灶结节性增生的临床与病理学表现分析

目的分析肝脏局灶结节性增生的临床和病理学表现。方法随机选取2010年10月～2012年10月期间收救治的60例肝脏局灶结节性增生患者,对其中30例进行手术切除,回顾分析其临床和病理学表现。结果本组患者26例无明显症状,34例有右上腹隐痛或闷胀不适症状;彩色多普勒超声检查可见中央瘢痕处搏动的血流。CT检查呈现等密度或低密度包块,少数病变呈现低密度中央瘢痕。在增强扫描时,病灶迅速强化,在动脉期呈现中央低密度的星状瘢痕为其特异性表现;在病理学上,病灶边界清晰,呈分叶形状,切面颜色有灰白色、淡黄色或黄白色。在镜下,纤维结缔增生组织对大量结节状排列的正常肝细胞进行包绕,纤维组织、畸形血管于病变内部形成星芒状的瘢痕,由病变内部向外延伸,病变内部不含有正常的中央静脉和门静脉分支。结论通过影像学检查,可对肝脏局灶结节性增生作出诊断,对排除恶性肿瘤困难或病灶在短期内增大的患者应首选外科手术治疗。     

Atypical hepatic focal nodular hyperplasia presenting as acute abdomen and misdiagnosed as hepatocellular carcinoma

Focal nodular hyperplasia (FNH) is a rare benign hepatic lesion, which is usually asymptomatic and solitary. Complications such as rupture and bleeding are extremely rare and only five cases have existed since 1975. We report a case of a 26-year-old woman with spontaneous rupture and hemorrhage of huge FNH presenting as acute abdomen. Different to previously recorded cases, this case was concomitant with multiple hepatic adenomas, which was misdiagnosed as rupture of hepatocellular carcinoma (HCC) with multiple intrahepatic spreading in another hospital. Our case highlights thepossible association between the size of FNH and the risk of rupture, and emphasizes the need to consider this in making treatment decisions. Although a conservative approach for asymptomatic FNH is well established, the potential for surgical intervention should always be considered, especially for large FNH. We recommend surgical resection of large FNH (>5 cm), symptomatic or not, rather than observation.     

Oral contraceptive-dependent growth of focal nodular hyperplasia

Abstract A 22 year old woman was incidentally found to have a hepatic small haemangioma-like mass, measuring 1.4 cm in diameter, by an ultrasonographic examination. The mass demonstrated no change in size or appearance for 6 months until the patient began to take oral contraceptives. Eventually, the mass increased to 2.0 cm in diameter after using oral contraceptives for 6 months. A histological examination suggested the mass to be typical focal nodular hyperplasia, and not hepatic adenoma. There was no further change in either size or appearance in the ensuing 1 year after the discontinuation of oral contraceptives.     

Atypical focal nodular hyperplasia of the liver

BACKGROUND:Focal nodular hyperplasia,a benign hepatic tumor,is usually asymptomatic.However,rarely the entity can cause symptoms,mandating intervention. METHOD:We present a case of focal nodular hyperplasia of the liver,which caused a considerable diagnostic dilemma due to its atypical presentation. RESULTS:A 29-year-old woman presented with a 15-year history of a progressively increasing mass in the right upper quadrant which was associated with pain and emesis. Examination showed a firm,mobile mass palpab...