Treatment is focused on its underlying conditions and com. Nodular regenerative hyperplasia nrh is an uncommon liver disease characterised histologically by numerous small hyperplastic nodules that are not separated by fibrotic tissue. Comparison of computed tomography features between follicular. Jan 08, 2019 nodular regenerative hyperplasia nrh is a rare liver disease in which normal liver tissue transforms into multiple, small clusters nodules of replicating liver cells regenerating hepatocytes. Pasd shows proliferated blood vessels in center of scar with minimal inflammation ll 200x. Mr imaging of hepatic nodular regenerative hyperplasia. Right axial ct section of the liver in the same patient shows no evidence of fibrosis or focal lesions.
This study aimed to compare ct features of follicular neoplasm and nodular hyperplasia and to determine the specific ct features that. Benign hepatic nodular lesions after treatment for childhood. To investigate nodular regenerative hyperplasia nrh as a vascular complication of systemic sclerosis ssc with microvasculopathy as a common denominator. Nodular hyperplasia can refer to a number of things.
There was no evidence of cirrhosis or cholestasis or steatosis. Nodular transformation nodular regenerative hyperplasia of the liver. Nodular regenerative hyperplasia nrh of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. Focal liver lesions, most commonly nodular regenerative hyperplasia, are an important clue to the underlying condition. Department of diagnostic radiology, paediatric radiology, university of cologne medical school, kerpener strasse 68, d50924 cologne, germany. Jan 12, 2015 hepatic focal nodular hyperplasia fnh is a nonmalignant condition rarely affecting children previously treated for cancer, especially those who received hematopoietic sct hsct. Various systemic diseases and drugs are associated with nrh, such as lymphoproliferative syndromes, chronic vascular disorders, rheumatologic disorders, steroids, and antineoplasticmedication. Liver and intrahepatic bile ducts tumor nodular regenerative. Focal nodular hyperplasia fnh is a regenerative mass lesion of the liver and the second most common benign liver lesion most common is a hemangioma.
Nodular regenerative hyperplasia nrh of the liver is a condition characterized by multiple monoacinar regenerative nodules in the absence of fibrous septa. Portal hypertension, nodular regenerative hyperplasia of the. Focal nodular hyperplasia and hepatocellular adenomas. Smi is a software function in a toshiba aplio 500 system. Regenerative nodular hyperplasia rnh represents the endstage of vascular lesions of the liver induced by chemotherapy. Very rare, focal form of nodular regenerative hyperplasia, usually perihilar region portal hypertension usually prominent due to portal venous thrombosis. Nodular regenerative hyperplasia nrh of the liver is a rare and poorly understood liver disease. It is important to consider not only malignant liver lesions, but also benign solid and cystic liver lesions such as hemangioma, focal nodular hyperplasia, hepatocellular adenoma, and hepatic cysts, in the differential diagnosis. Focal nodular hyperplasia radiology reference article. Imaging diagnosis of earlystage nrh remains problematic.
Nodular regenerative hyperplasia definition of nodular. Jan 04, 2016 increasingly, focal nodular hyperplasia fnh is being recognized as an incidental finding, owing to the widespread use of diagnostic imaging for unrelated conditions. Nodular regenerative hyperplasia and portal hypertension in a. The increasing and widespread use of imaging studies has led to an increase in detection of incidental fll. It can develop in people of all ages but is more common in older adults. Nrh of the liver is an established pathologic entity that should not be confused with focal nodular hyperplasia, hepatocellular adenoma, or the regenerative nodules associated with cirrhosis. Supported by the intramural research programs of the national institutes of.
Micronuodular transformation nodular regenerative hyperplasia of the liver. It is a form of liver disease characterized by mostly preserved liver function, with signs of portal hypertension through clinical, radiologic, or endoscopic evaluation. Pathology outlines nodular regenerative hyperplasia. Focal nodular hyperplasia harvard catalyst profiles. In a pathologic study of explanted livers, krasinskas et al reported fnhlike lesions in two of 16 patients. This report describes a 64yearold man with clinically presumed cirrhosis who presented to our institution with coffeeground emesis, esophageal varices, ascites, and encephalopathy. Many fnhs have characteristic radiographic features on multimodality imaging, but some lesions may be atypical in appearance. Imaging feature so diffuse nodular regenerative hyperplasia of liver with portal hypertension. L, nodus, knot of a structure or mass small, firm, and knotty. Focal nodular hyperplasia multiple left axial cect in a 52yearold man with a renal transplant shows massive ascites and esophageal varices. However, distinction is critical because their clinical significance is quite different.
Nodular regenerative hyperplasia nrh is the most common pathologic liver. Ct and mr imaging findings in focal nodular hyperplasia of. Nodular regenerative hyperplasia nrh and large regenerative nodules lrn are distinct types of hepatocellular nodules that have. Nodular regenerative hyperplasia nrh and large regenerative nodules lrn are distinct types of hepatocellular nodules that have been confused in the radiology literature.
Pdf nodular regenerative hyperplasia nrh is an uncommon liver disease characterised. To date, appropriate management for bethesda iv thyroid nodules is controversial, and no specific features of follicular neoplasm and nodular hyperplasia on ultrasonography, computed tomography ct, or other imaging modalities have been reported. This lesion was subsequently termed nodular regenerative hyperplasia by steiner,12 who described regenerative liver. This pictorial essay covers imaging characteristics in abdominopelvic region. Focal nodular hyperplasia is defined as a nodule composed of benignappearing hepatocytes occurring in a normal or nearly normal liver. Nodular regenerative hyperplasia nrh has also been reported as a rare sequela of antineoplastic therapy. Distinguishing clinical and imaging features of nodular. Efficacy comparison of multiphase ct and hepatotropic. Nodular regenerative hyperplasia is a form of liver hyperplasia associated with portal hypertension. This study aimed to compare ct features of follicular neoplasm and nodular hyperplasia and to determine the specific ct features that could be used. Nodular regenerative hyperplasia nrh is a rare liver condition.
Although atypical imaging features are the exception rather than the rule, it is sometimes difficult to differentiate focal nodular hyperplasia lacking. Focal nodular hyperplasia is a descriptor in the national library of medicines controlled vocabulary thesaurus, mesh medical subject headings. Usefulness of power doppler imaging in the diagnosis of small. Nodular hyperplasia diffusely affecting entire liver but with no minimal fibrous septa partial nodular transformation. Hepatic focal nodular hyperplasia fnh is a nonmalignant condition rarely affecting children previously treated for cancer, especially those who. Trichrome shows fibrous scar with vesselsbile ductules ul 40x. This report describes 21 cases of nodular regenerative hyperplasia nrh and its clinical and radiologic features. We report the case of a 71yearold patient with celiac disease and unexplained portal hypertension. It is thought to be the result of obliterative vasculopathy, and it has. We evaluated the usefulness of power doppler imaging pdi compared with conventional doppler imaging cdi for the diagnosis of small focal nodular hyperplasia of the liver fnh prospectively. Lack of perinuclear collagen tissue distinguishes nrh from typical regenerative nodules in the cirrhotic liver. The patient did not suffer from an autoimmune, rheumatologic. Focal nodular hyperplasia fnh is the second most benign hepatic tumor which both male and female,affects but predominantly women 80% 95% during thirdfifth decade of life 1 2.
Pasd shows tortuous bile ductules at edge of scar with minimal inflammation ur 200x. Hyperdense or hyperintense due to fibrous tissue gadoxetateenhanced mr most specific test to diagnose fnh prolonged enhancement of entire fnh except scar on delayed scan top differential diagnoses hepatic adenoma rarely retains gadoxetate on delayed phase mr fibrolamellar. Sonazoid is contrastmaterial for us and it is a intervention for patient with fnh. Nodular regenerative hyperplasia in common variable. Jul 31, 20 nodular regenerative hyperplasia nrh of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. Nodular regenerative hyperplasia pathology outlines. Nodular regenerative hyperplasia an overview sciencedirect. Three patients at two institutions were identified who had both abdominal mr imaging and pathologic evidence of nrh. This report describes a 64yearold man with clinically presumed cirrhosis who presented to our institution with coffeeground emesis, esophageal varices, ascites, and.
Superb microvascular imaging in focal nodular hyperplasia. For imaging of the right upper quadrant, ultrasonography us is more widely used than other modalities. Nodular regenerative hyperplasia nrh, a rare condition that is commonly associated with noncirrhotic portal hypertension, is not well described in the mr literature. Nodular regenerative hyperplasia nrh of the liver is often associated with rheumatologic or lymphoproliferative disorders and a cause of portal hypertension in some patients.
Biopsy of the liver revealed nrh as the underlying cause. Atypical ct and mri features of focal nodular hyperplasia. Although some patients are successfully managed medically or with shunting. Ames jt, federle mp, chopra k 2009 distinguishing clinical level settings, potentially hampering readers perception of and imaging features of. Two liver lesions hemangioma and focal nodular hyperplasia. Nodular regenerative hyperplasia of the liver nrh, characterized by multiple hepatic nodules in the absence of fibrosis, is a rare but important complication of systemic lupus erythematosus sle associated with noncirrhotic portal hypertension. Other benign liver lesions such as hepatocellular adenoma and hemangioma have been etiologically associated with the use of medications intensively used in pediatric oncology, such as steroids and oral contraceptives 1115.
Nodular regenerative hyperplasia nrh is described as a rare form of noncirrhotic portal hypertension in the absence of a recognizable cause. Regenerative nodular hyperplasia of the liver related to. New imaging techniques, such as triple phase spiral ct and fast mr imaging, have markedly increased the detection of focal nodular hyperplasia, the second most common benign hepatic tumor. In our series, hypervascular lesions sharing all the features of fnhlike lesions were seen in 14% of. When these nodules become confluent they may be seen with sonography or ct. The aim of our study was to evaluate the role of gadoxeticacid mri in the differentiation of small. Significance of magnetic resonance imaging in the diagnosis.
Nodular regenerative hyperplasia nrh, characterized by diffuse hepatic micronodular transformation in groups without fibrous septa between the nodules, is a rare benign liver lesion that has many synonyms in previous literature. Nodular regenerative hyperplasia is a common histologic finding in patients with opv, while liver nodules are rarely described. Liver transplantation for the treatment of nodular. Nodular regenerative hyperplasia nrh is an increasingly recognized form of noncirrhotic portal hypertension ncph.
Four patients with five fnh lesions, which had been detected incidentally, were diagnosed histologically. Edmondson, 1 introduced the term focal nodular hyperplasia fnh in 1958. Levels of protein s activity were lower among patients with hivassociated nodular regenerative hyperplasia when compared with hivpositive patients without nodular regenerative hyperplasia and when compared with hivnegative patients with nodular. Pathologic evaluation is the mainstay of accurate diagnosis. The resulting image revealed that nodules were well perfused and. Levels of protein s activity were lower among patients with hivassociated nodular regenerative hyperplasia when compared with hivpositive patients without nodular regenerative hyperplasia and when compared with hivnegative patients with nodular regenerative hyperplasia p imaging pdi compared with conventional doppler imaging cdi for the diagnosis of small focal nodular hyperplasia of the liver fnh prospectively. Nodular liver regeneration definition of nodular liver. Grossly, the liver in massive hepatic necrosis is soft, not diffusely firm as in. Liver biopsy showed diffuse nodular regenerative hyperplasia. Nodular regenerative hyperplasia nrh of the liver is a condition characterized by diffuse micro or macronodular transformation of the hepatic parenchyma without formation of fibrous septa betweenthe nodules. Pseudotumoral presentation of nodular regenerative. Nrh may lead to the development of noncirrhotic portal hypertension.
The aim of this study was to compare the efficacy of contrastenhanced ct and mri in the diagnosis of fnh. Get another captcha get an audio captcha get an image captcha help. Diagnosing nodular regenerative hyperplasia of the liver is. Nrh is histologically defined by diffuse micronodular transformation without fibrous septa. Nodular regenerative hyperplasia regeneration of a skin condition characterized by crops of small, tender, erythematous nodules on the legs has been reported by people with hiv infection, crohns disease, colon cancer, breast cancer, preventive health care. Will you have nodular regenerative hyperplasia with. The characterization of small lesions in cirrhotic patients is extremely difficult due to the overlap of imaging features among different entities in the stepway of the hepatocarcinogenesis. Background and aims nodular regenerative hyperplasia nrh of the liver is associated with several diseases and drugs. Nodular regenerative hyperplasia nrh is the leading cause of noncirrhotic portal hypertension in western countries. Nodular regenerative hyperplasia nrh of the liver is an uncommon condition. Nov 11, 2016 the characterization of small lesions in cirrhotic patients is extremely difficult due to the overlap of imaging features among different entities in the stepway of the hepatocarcinogenesis.
Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity. Nodular regenerative hyperplasia nrh is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules. Liver biopsy showed a fine nodular liver parenchyma with hepatocyte ballooning and nodular arrangement without fibrous septae. Patients with focal nodular hyperplasia will undergo ultrasonography with superbmicrovascular imaging and additional sonazoidenhanced ultrasonography. Different clinical behaviour influences the importance of differentiating focal nodular hyperplasia fnh from other focal liver lesions flls. Nodular regenerative hyperplasia genetic and rare diseases. Hypodense or isodense to normal liver delayed scans mass. Definition general clinical features laboratory gross description microscopic histologic description microscopic histologic images. The significance of magnetic resonance imaging in the diagnosis of nrh is still. The goal was to evaluate its incidence and impact on the outcome of patients resected for colorectal liver metastases clm. Nodular regenerative hyperplasia aasld journals wiley.