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Background

This is an uncommon benign tumor of the liver that may be mistaken for a malignancy.The pathologists plays a critical role in making the tissue diagnosis.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS  
INCIDENCE/
PREVALENCE
 
AGE Child bearing age
SEX Female predominance
GEOGRAPHY  
EPIDEMIOLOGIC ASSOCIATIONS  
ORAL CONTRACEPTIVES  
Oral contraceptives and focal nodular hyperplasia of the liver.

Hagay ZJ, Leiberman RJ, Katz M, Witznitzer A.

Division of Obstetrics and Gynecology, Soroka University Hospital, Beer-Sheva, Israel.

Arch Gynecol Obstet. 1988;243(4):231-4. Abstract quote  

A variety of benign liver tumors associated with the use of oral contraceptives has been described. However, there is controversy regarding the possible relation of focal nodular hyperplasia of the liver to oral contraceptive therapy. Over a ten-year period at the Soroka Medical Center, two young women were found to have hepatic tumors diagnosed as focal nodular hyperplasia. In both cases the hepatic nodules were an incidental finding at laparotomy and were thought to be metastatic tumors. The clinical and pathological findings in both cases are reported. The features of focal nodular hyperplasia and its possible relation to oral contraceptive use is discussed.

PIP: The controversy concerning the possible relation of focal nodular hyperplasia (FNH) of the liver to oral contraceptives (OCs) continues. Sex steroids, such as estrogen, affect the liver more than any other organ outside of the genital organs. Research shows that in 10-40% of women taking OCs the bile secreting function of hepatocytes is impaired. Even though most liver cell adenomas in young women have been associated with OC use, this has not necessarily been the case for FNH. In addition, many studies demonstrate that FNH does not progress to become hepatic carcinoma, and FNH has never been fatal. Despite the fact that FNH mainly occurs in women, it has been present in newborn infants and in the elderly. Moreover, there is no evidence that using OCs increases the frequency of FNH. 2 cases of FNH at a medical center in Beer-Sheva, Israel only add to this controversy. A 21 year old, unmarried woman had been taking OCs 4 years prior to her arrival at the hospital. Complaining of 2 weeks of pain in the left chest and in the left upper quadrant of the abdomen and upon completion of a physical exam, surgery was performed and revealed a primary Ewing's sarcoma of the bone marrow of her lower left ribs. The liver appeared normal except for a 3x2x2 cm FNH on the anterior surface of the left lobe. She died 1 month later from respiratory arrest. A 32 year old woman who did not use OCs indicated pain for 6 hours in the left lower quadrant of the abdomen. Practitioners found the left Fallopian tube and ovary gangrenous and twisted. In addition, they found multiple FNHs on the liver. A total hysterectomy, bilateral salpingo-ovariectomy, and a liver biopsy were performed. She recovered with no complications.

 

DISEASE ASSOCIATIONS CHARACTERIZATION

 

PATHOGENESIS CHARACTERIZATION
GENERAL  
Etiological analysis of focal nodular hyperplasia of the liver, with emphasis on similar abnormal vasculatures to nodular regenerative hyperplasia and idiopathic portal hypertension.

Kondo F, Nagao T, Sato T, Tomizawa M, Kondo Y, Matsuzaki O, Wada K, Wakatsuki S, Nagao K, Tsubouchi H, Kobayashi H, Yasumi K, Tsukayama C, Suzuki M.

Department of Pathology, Chiba University School of Medicine, Japan.

Pathol Res Pract. 1998;194(7):487-95. Abstract quote  

Pathological studies were performed on 23 cases of focal nodular hyperplasia (FNH) under the hypothesis that FNH is a hyperplastic lesion caused by abnormal vasculatures of portal tracts within the nodule.

For a comparison of the histological features of portal tracts, nodular regenerative hyperplasia (NRH), idiopathic portal hypertension (IPH), chronic hepatitis and so-called normal liver were used as control tissues. Extranodular areas of FNH nodules were also examined.

Clinical data were briefly summarized. Most of the portal tracts within FNH nodules showed various abnormal findings, such as dilatation and/or stenosis of portal vein, muscular thickening of arterial wall with dilated or stenotic lumina, lymphocyte infiltration, and bile ductule proliferation. However, portal vein thrombi were not found. These findings were not thought to represent compensatory reaction to portal vein thrombosis. Similar abnormal features were also observed in extranodular areas of FNH although to a milder degree. These abnormal features resembled those of NRH and IPH. Moreover, the characteristic scar-like tissues within FNH nodules were proved to be abnormally large portal tracts including large feeding arteries, portal veins and bile ducts. It has been believed that septa and scar-like tissue within FNH nodules are not portal tracts and that arterial malformation independent of portal tracts are related to the development of FNH. In addition, venous structures within FNH modules have until now not been considered to be portal veins. However, this study revealed that severe anomaly of portal tracts including portal veins and hepatic arterial branches existed in FNH nodules. Moreover, portal tracts in extranodular areas were also abnormal.

Clinically, only one patient had a history of oral contraceptives. Based on these findings, congenital anomaly of the portal tracts histologically resembling the abnormal portal tracts of NRH and IPH may be related to the pathogenesis of FNH.

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
Focal nodular hyperplasia: findings at state-of-the-art MR imaging, US, CT, and pathologic analysis.

Hussain SM, Terkivatan T, Zondervan PE, Lanjouw E, de Rave S, Ijzermans JN, de Man RA.

Department of Radiology, Erasmus Medical Center, Dr Molewaterplein 40, 3015 GD Rotterdam, the Netherlands.
Radiographics. 2004 Jan-Feb;24(1):3-17; discussion 18-9. Abstract quote  

Focal nodular hyperplasia (FNH) is the second most common benign liver tumor after hemangioma. FNH is classified into two types: classic (80% of cases) and nonclassic (20%). Distinction between FNH and other hypervascular liver lesions such as hepatocellular adenoma, hepatocellular carcinoma, and hypervascular metastases is critical to ensure proper treatment.

An asymptomatic patient with FNH does not require biopsy or surgery. Magnetic resonance (MR) imaging has higher sensitivity and specificity for FNH than does ultrasonography or computed tomography.

Typically, FNH is iso- or hypointense on T1-weighted images, is slightly hyper- or isointense on T2-weighted images, and has a hyperintense central scar on T2-weighted images. FNH demonstrates intense homogeneous enhancement during the arterial phase of gadolinium-enhanced imaging and enhancement of the central scar during later phases. Familiarity with the proper MR imaging technique and the spectrum of MR imaging findings is essential for correct diagnosis of FNH.
Radiological features of focal nodular hyperplasia of the liver in children.

Cheon JE, Kim WS, Kim IO, Jang JJ, Seo JK, Yeon KM.

Department of Radiology and the Institute of Radiation Medicine, College of Medicine, Seoul National University, Seoul, Korea.


Pediatr Radiol. 1998 Nov;28(11):878-83. Abstract quote  

BACKGROUND: Focal nodular hyperplasia (FNH) is an unusual hepatic tumour in children and should be distinguished from other hepatic lesions.

OBJECTIVE: To describe the imaging characteristics of FNH in children.

MATERIALS AND METHODS: We examined five patients (three boys and two girls, mean age 9.4 years) with pathologically confirmed FNH. The diagnosis was obtained by tumour resection (n = 4) and percutaneous needle biopsy (n = 1). One patient with multiple FNHs showed recurrent lesions after tumour resection. All patients were studied with US (including colour and power Doppler US [n = 3]) and CT. Dynamic enhanced CT scans were available in three patients. MRI (n = 2) or coeliac angiography (n = 1) was performed in three patients.

RESULTS: Seven of eight FNH lesions in five patients were demonstrated by imaging. The average size of the lesions was 6.5 cm. Six lesions detected on US showed variable echogenicity with a central hyperechoic scar (n = 2). On Doppler examination, central or peripheral hypervascular areas were seen (n = 3). Six lesions detected on contrast-enhanced CT showed high attenuation (n = 4) or iso-attenuation (n = 2). On early phase scans, all the lesions (n = 3) showed high attenuation. Irregular linear or ovoid central scars were detected in two patients on CT. MR demonstrated three lesions in two patients, one of which had not been detected by US or CT. A central low signal intensity scar (n = 1) was seen on T2-weighted MRI. Coeliac angiography performed in one patient showed a hypervascular mass with homogeneous staining.

CONCLUSION: FNH in children shows a wide spectrum of imaging findings on various radiological examinations and the typical central scar was not always seen on imaging studies. Dynamic enhanced CT obtained in the early phase and colour Doppler studies may be helpful in the diagnosis of FNH by allowing characterisation of tumour vascularity. FNH should be included in the differential diagnosis of liver mass in children.
Focal nodular hyperplasia of the liver: radiologic-pathologic correlation.

Buetow PC, Pantongrag-Brown L, Buck JL, Ros PR, Goodman ZD.

Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

Radiographics. 1996 Mar;16(2):369-88. Abstract quote  

Focal nodular hyperplasia (FNH) is a benign hepatic tumor that likely represents a local hyperplastic response of hepatocytes to a congenital vascular anomaly. It is most commonly seen in middle-aged women and is typically a solid mass measuring less than 5 cm in diameter.

Most lesions have central scars that contain thick-walled vessels that provide excellent arterial blood supply; hemorrhage, necrosis, and infarction are, therefore, extremely unusual. Characteristic imaging features include a hypervascular homogeneous tumor with a central scar and with both hepatocellular and reticuloendothelial function. Ultrasonography, computed tomography, scintigraphy, and magnetic resonance imaging all offer different advantages in the detection and characterization of FNH.

There is excellent correlation between the pathologic and imaging features of FNH. In many cases, it is possible to obtain a prospective imaging diagnosis of FNH; however, in some cases, the distinction between FNH and other primary hepatic neoplasms is not possible. In these latter cases, close imaging follow-up, needle biopsy, or even surgical resection may be necessary.
Contribution of CT to characterization of focal nodular hyperplasia of the liver.

Procacci C, Fugazzola C, Cinquino M, Mangiante G, Zonta L, Andreis IA, Nicoli N, Pistolesi GF.

Department of Radiology, University Hospital, Verona, Italy.

Gastrointest Radiol. 1992 Winter;17(1):63-73. Abstract quote  

Our personal series of 20 cases of focal nodular hyperplasia (FNH) of the liver is presented.

All lesions were studied with computed tomography (CT), 16 of which with surgical control. Retrospective evaluation of the CT features of the identified FNH, along with those of five hepatocellular adenomas (HCA) and 30 hepatocellular carcinomas (HCC), allowed the definition of specific patterns leading to a correct characterization of FNH in 78% of cases.

This greatly reduced the diagnostic errors, with the sole exception of patients with fatty liver in whom nuclear medicine may eventually provide a correct characterization. Fine-needle biopsy is thus only necessary in the dubious cases. A precise diagnostic workup of FNH is necessary, since it may avoid the surgical intervention.
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
Focal nodular hyperplasia of the liver: a clinicopathologic study and review of the literature.

Knowles DM, Wolff M.

Hum Pathol. 1976 Sep;7(5):533-45 Abstract quote.  

We received the clinical records and pathologic material of 20 patients with biopsy proven hepatic focal nodular hyperplasia.

The majority of the patients were females of child bearing age, five of whom had a history of oral use of contraceptives. In every instance focal nodular hyperplasia was an incidental finding; liver function tests were always normal. Focal nodular hyperplasia is a distinct histopathologic entity, distinguishable from liver cell adenoma. Specifically it consists of nodular aggregates of cytologically normal hepatocytes with foci of intranodular bile duct proliferation. Focal nodular hyperplasia appears to be a benign entity, even in patients in whom the lesion was not excised. The association between focal nodular hyperplasia and oral use of contraceptives may be coincidental, although hormonally related vascular changes may be responsible for rupture of the lesion.

PIP: The clinical records and biopsy proven material from 20 cases of focal nodular hyperplasia were reviewed, and the English literature on the subject was surveyed. Most of the 20 patients were women of childbearing age. A history of oral contraceptive use was obtained in 5 cases. 3 of the patients were males. The condition was an incidental finding in all 20 cases. Liver function tests were normal. The histopathologic findings are distinguishable from liver cell adenoma. Nodular aggregations of normal hepatocytes with foci of intranodular bile duct proliferation were seen. The nodules blended into the surrounding liver parenchyma while liver cell adenomas are
encapsulated masses without the central scar or radiating fibrous septa. Figures illustrate histologic findings of focal nodular hyperplasia. A review of the English literature revealed only 82 acceptable cases of focal nodular hyperplasia, 12 of which were necropsy findings or lacked clinical data. A few cases have been among males. The association with use of oral contraceptives may be only coincidental. Only 29 of the 82 were 18 years of age or older, 2 of whom were women. Mean age of adult males was 42 years and of adult females, 34 years. In 16 patients the focal nodular hyperplasia had been an incidental finding but in 13 there had been either an abdominal mass or symptoms of abdominal discomfort, nausea, and vomiting.

Focal nodular hyperplasia is a benign lession. Removal is unnecessary except in large tumors. It is possible that hormonally related vascular changes may lead to rupture of the lesion with intraabdominal hemorrhage.
VARIANTS  

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  
Diagnosis of focal nodular hyperplasia of the liver by needle biopsy.

Makhlouf HR, Abdul-Al HM, Goodman ZD.

Division of Hepatic Pathology, Department of Hepatic and Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

Hum Pathol. 2005 Nov;36(11):1210-6. Abstract quote  

Focal nodular hyperplasia (FNH) of the liver can be a difficult diagnosis to establish in limited diagnostic samples such as a needle-core tissue biopsy, especially for pathologists with limited experience with the lesion.

To characterize the features that can be used to make the diagnosis, we reviewed and analyzed the clinicopathologic features of 100 consecutive cases submitted for consultation in which we were confident of the diagnosis of FNH in needle biopsy material. A diagnosis of FNH was correctly made by the contributing pathologist in 24 of the 100 referred cases. Most of the patients (81%) were women of childbearing age with a mean age of 36.75 +/- 9.82 years. Most of the patients (70%) were asymptomatic at diagnosis.

The most consistent diagnostic histological feature of FNH in needle biopsy was the presence of ductular reaction with varied intensity at the junction of the fibrous septa with the hepatocellular component, which was present in all 100 cases. Thick abnormal arteries were seen in all but 2 cases (n = 98). Features of chronic cholestasis with cholate stasis and accumulation of copper (demonstrable by the rhodanine stain) and copper-binding protein (demonstrable with the Victoria blue stain) were nearly as common (n = 94).

A confident diagnosis of FNH can be made with a needle biopsy, especially if the biopsy is known to come from a mass, and the lesion contains characteristic fibrosis with ductules at the interface between hepatocytes and the fibrous region, prominent arteries, and benign hepatocytes with features of chronic cholestasis.
Focal nodular hyperplasia of the liver: pathological analysis of 11 cases.

Pan Y, Wang ZM, Mou LJ, Teng XD, Zheng ZJ, Ying LX.

Department of Pathology, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.
Hepatobiliary Pancreat Dis Int. 2004 May;3(2):199-203. Abstract quote  

BACKGROUND: Focal nodular hyperplasia (FNH) is a benign tumor-like lesion of the liver, predominantly affecting women. Its etiology is obscure and its pathogenesis is poorly understood. FNH should be differentiated from other benign and malignant hepatic lesions. The aim of this study was to explore the pathological characteristics of FNH of the liver.

METHODS: Eleven patients with FNH were studied retrospectively by using hematoxylin and eosin, immunohistochemical and histochemical staining.

RESULTS: In 8 female and 3 male FNH patients aged 19 to 54 years (mean 32), most of lesions showed central scars macroscopically. Microscopically 8 patients were found of classical type, 2 were of telangiectic type, and 1 was of mixed type.

CONCLUSION: FNH is an uncommon benign hyperplastic lesion of the liver. It should be differentiated from hepatocellular adenoma, alpha-fetoprotein negative hepatocellular carcinoma, and fibrolamellar carcinoma.
Histologic scoring of liver biopsy in focal nodular hyperplasia with atypical presentation.

Fabre A, Audet P, Vilgrain V, Nguyen BN, Valla D, Belghiti J, Degott C.

Service d'Anatomie Pathologique, Hopital Beaujon, Clichy, France.

Hepatology. 2002 Feb;35(2):414-20 Abstract quote.  

The contribution of radio-guided transcutaneous biopsy in the diagnosis of focal nodular hyperplasia (FNH) of the liver was compared with the findings on surgical specimens to assess its contribution in clinical and radiologic atypical cases.

This retrospective study involved 30 patients with atypical tumors on imaging who underwent liver biopsy and then surgery. All surgical specimens were diagnosed as FNH, either classical (n = 18) or nonclassical (n = 12). Imaging data were reviewed according to 4 radiologic criteria on magnetic resonance imaging (MRI) and/or computed tomography (CT) scan (hypervascularity, homogeneity, nonencapsulation, and presence of a central scar), and classified depending on the number of criteria found (group I, 4 of 4; group II, 3 of 4; group III, 2 or fewer).

Histologic assessment of ultrasound (US)-guided liver biopsy recorded major diagnostic features (fibrous bands, thick-walled vessels, reactive ductules, and nodularity) and minor features (sinusoidal dilatation and perisinusoidal fibrosis). "Definite FNH" (3 or 4 major features) was diagnosed in 14 biopsies, "possible FNH" (2 major and 1 or 2 minor features) in 7 cases, and "negative for FNH" (2 or fewer major features without minor features) in 9 cases. The diagnosis of FNH on biopsy was reached in 14 cases (58.3%) in patients with 2 or fewer imaging criteria (group III; n = 24). Biopsies with a diagnosis of "possible FNH" corresponded to a large proportion of telangiectatic-type FNH on the specimen.

In conclusion, liver biopsy does not appear to be necessary in cases in which imaging is typical. However, the absence of radiologic diagnostic criteria in FNH does not preclude a positive diagnosis on liver needle biopsy. Using the proposed histologic scoring system, surgical management may be avoided in these cases.
FNH-like nodules: Possible precursor lesions in patients with focal nodular hyperplasia (FNH).

Lepreux S, Laurent C, Balabaud C, Bioulac-Sage P.

Service de Hepato-gastroenterologie, Hopital St Andre, CHU Bordeaux, France.
Comp Hepatol. 2003 Jun 26;2(1):7. Abstract quote  

BACKGROUND: The typical lesion of focal nodular hyperplasia (FNH) is a benign tumor-like mass characterized by hepatocytic nodules separated by fibrous bands. The solitary central artery with high flow and the absent portal vein give the lesions their characteristic radiological appearance. The great majority of cases seen in daily practice conform to the above description. Additional small nodules (from 1-2 up to 15-20 mm in diameter) detected by imaging techniques or on macroscopic examination may be difficult to identify as representing FNH if they lack the key features of FNH as defined in larger lesions. The aim of this study was to characterize these small nodules, and to compare their characteristics with those of typical lesions of FNH present in the same specimens.

RESULTS: Eight patients underwent hepatic resections for the removal of a mass lesion ("nodule") diagnosed as: FNH (1 patient); nodules of unknown nature (5 patients); or nodules thought to be adenoma or hepatocellular carcinoma (2 patients). Six nodules out of 9 discovered by imaging techniques met histopathological criteria for the diagnosis of typical FNH, at least in parts of the nodule; 2 nodules corresponded to a minor form of FNH ("subtle FNH") and one nodule to a steatotic area. Although FNH was thought to be found in a normal or nearly normal liver, this study revealed that, in addition, there were various types of small FNH-like nodules and vascular abnormalities in the liver with typical FNH nodule. The various types of small FNH-like nodules (n = 8, diameter 2 to 20 mm) consisted of the association to various degrees of numerous and/or enlarged arteries in portal tracts or in septa, with hyperplastic foci, slight ductular reaction, and regions of sinusoidal dilatation, accompanied by thin fibrous bands. Vascular abnormalities consisted of unpaired arteries, portal tracts with arteries larger than the associated bile duct, and regions of sinusoidal dilatation.

CONCLUSIONS: Although these small nodules can be considered as insufficient type or abortive forms of FNH, or adenoma, they can be precursors of the large mass lesions in which FNH was recognized and defined.
ATYPIA  
Severe cytological atypia (large cell change) in focal nodular hyperplasia with numerous mallory bodies. A benign (adaptive) change?

Agaimy A, Kaiser A, Wuensch PH.

Institute of Pathology, Clinical Center of Nuremberg, Germany.


Pathol Res Pract. 2003;199(7):509-11. Abstract quote  

Focal nodular hyperplasia (FNH) is a benign hepatocellular lesion composed of hyperplastic appearing hepatocytes arranged in nodules separated by fibrous septa that usually form a central stellate scar. Rare lesions that show unusual cytological or architectural features were reported as variants of focal nodular hyperplasia.

We present the morphological features of a case of FNH with severe cytological atypia (so-called large cell change) in a 73-year-old man. In addition to diffuse cytological atypia, Mallory hyaline bodies were found in almost all lesional cells. This rare variant of FNH should be differentiated from other neoplastic lesions, in particular from the fibrolamellar variant of hepatocellular carcinoma.
CHOLESTASIS  
Cholestatic features in focal nodular hyperplasia of the liver.

Butron Vila MM, Haot J, Desmet VJ.

Liver. 1984 Dec;4(6):387-95. Abstract quote  

Twenty specimens of focal nodular hyperplasia were studied with special attention to the histological features of chronic cholestasis (grouped under the headings of cholestasis, cholate-stasis and signs of ductular reabsorption).

In all specimens, evidence was found for one or more features of cholestasis and cholate-stasis. Signs of ductular reabsorption were less constant, and apparently varied according to the developmental stage of the lesion. The cholestatic features emphasize the bile secretory capacity of the lesional parenchyma, and are apparently due to the lack of real bile ducts in the portal tract equivalents of the lesional tissue. Evidence is presented that the "ductular component" in FNH is not due to proliferation of pre-existing ductules, but rather derives from ductular metaplasia of liver cell plates in zone 1 equivalents.

This metaplastic development of a ductular network may serve the function of reabsorbing the biliary constituents produced by the lesional parenchyma, leading to periductular inflammation and progressive fibrosis, thus producing an equivalent of biliary fibrosis and biliary cirrhosis.
SPINDLE CELLS  
Spindle cell fragments in focal nodular hyperplasia of the liver. A case report.

Krishnamurthy S, Nerurkar AY.

Division of Cytopathology, Department of Pathology, 8th Floor, Annexe Building, Tata Memorial Hospital, Dr. E. Borges Road, Mumbai 400 012, India.
Acta Cytol. 2002 May-Jun;46(3):582-4. Abstract quote  

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.
TELANGIECTATIC  
Clinical, morphologic, and molecular features defining so-called telangiectatic focal nodular hyperplasias of the liver.

Bioulac-Sage P, Rebouissou S, Sa Cunha A, Jeannot E, Lepreux S, Blanc JF, Blanche H, Le Bail B, Saric J, Laurent-Puig P, Balabaud C, Zucman-Rossi J.

Department of Pathology, Centre Hospitalier Universitaire de Bordeaux, France.

Gastroenterology. 2005 May;128(5):1211-8. Abstract quote  

BACKGROUND & AIMS: Telangiectatic focal nodular hyperplasia (TFNH) of the liver is generally believed to belong to the focal nodular hyperplasia (FNH) family. The aim of this study was to use molecular markers, in addition to morphologic features, to better characterize TFNH.

METHODS: Thirteen patients with TFNH were compared with 28 patients with FNH and 17 patients with hepatocellular adenoma. Full clinical and morphologic data were analyzed. Molecular markers included determination of clonality by examining the active X chromosome, genome-wide allelotyping, a search for hepatocyte nuclear factor 1alpha (HNF1alpha) mutations, and determination of ANGPT1/ANGPT2 transcript levels.

RESULTS: No clinical differences were evident between patients with TFNH and adenoma; in particular, bleeding was observed in 77% and 53% of the cases, respectively. Patients with TFNH were more likely to experience nodule recurrence and the presence of multiple nodules than those with either FNH or adenoma. All TFNH and adenoma samples that were available for analysis were monoclonal, in contrast to 40% of the FNH samples. Chromosome losses confirmed monoclonality and were significantly less frequent in TFNH and FNH (22% and 26%) than in adenoma (53%). HNF1alpha mutations were found exclusively in half of the adenomas. ANGPT2 was overexpressed in TFNH and down-regulated in adenoma (P < .01) and FNH (P < .0005).

CONCLUSIONS: TFNHs are monoclonal lesions frequently subject to bleeding that are similar to adenomas not carrying HNF1alpha mutations and require a similar type of treatment. However, morphologic and molecular data support the hypothesis that TFNH is a separate entity.
Telangiectatic focal nodular hyperplasia of the liver: a case detected at birth.

Kim HS, Kim YA, Kim CJ, Suh YL, Jang JJ, Chi JG.

Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.


J Korean Med Sci. 2003 Oct;18(5):746-50. Abstract quote  

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.
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To our knowledge, this is considered to be the first reported case of telangiectatic FNH detected at birth.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
HEPATIC ADENOMA  
Hepatic adenoma and focal nodular hyperplasia: a diagnostic dilemma.

Low V, Khangure MS.

Department of Diagnostic Radiology, Royal Perth Hospital, W.A.


Australas Radiol. 1990 May;34(2):124-30. Abstract quote  

Focal Nodular Hyperplasia (FNH) and Hepatic Adenoma (HA) remain difficult diagnostic problems due to their variable imaging appearances. Five new cases are presented, illustrating this variability, and the current literature is reviewed. Ultrasonography is a sensitive modality for their detection but is otherwise non-specific.

On computer tomography, the presence of a scar suggests FNH, whilst haemorrhage suggests HA. However these features are seen in only a small number of cases. Radionuclide (colloid) scanning aids considerably in FNH but a definitive role has not been found in HA. Angiography is helpful, providing anatomical information, sometimes diagnostic (septated blush in FNH, hypovascular areas in HA) and able to discern benign from malignant lesions.

Where imaging is not definitively diagnostic, percutaneous biopsy is indicated if FNH is suspected and surgical biopsy if HA is felt likely.

 

PROGNOSIS CHARACTERIZATION
REGRESSION  
Spontaneous regression of focal nodular hyperplasia of the liver.

Ohmoto K, Honda T, Hirokawa M, Mitsui Y, Iguchi Y, Kuboki M, Yamamoto S.

Division of Gastroenterology, Department of Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki 701-0192, Japan.

J Gastroenterol. 2002;37(10):849-53. Abstract quote  

Focal nodular hyperplasia (FNH) of the liver is a rare benign lesion that probably reflects a local hyperplastic response of hepatocytes to a vascular abnormality. Currently, the natural history of the disease remains largely unknown.

We present a patient with FNH of the liver who was followed up for 4 years. A 22-year-old woman with a 3-year history of oral contraceptive use was referred to our hospital in September 1996 for further examination of a liver tumor. A diagnosis of FNH was made using various imaging methods, such as ultrasonography, enhanced computed tomography (CT) scanning, MR imaging, and hepatic angiography, as well as fine-needle biopsy. A decrease in the size of the lesion was observed by enhanced CT scanning during the 4-year observation period. In this patient, oral contraceptive use and its discontinuation may have influenced the natural history of FNH.

The present case suggests that an accurate diagnosis is of the utmost importance, and a patient with FNH should be managed conservatively rather than by resection, because FNH has the potential for spontaneous regression with the discontinuation of oral contraceptives.

 

TREATMENT CHARACTERIZATION
GENERAL  
Laparoscopic resection of focal nodular hyperplasia.

Felsher J, Brody F.

Department of General Surgery, George Washington University, Washington, DC, USA.

Surg Laparosc Endosc Percutan Tech. 2003 Aug;13(4):276-9. Abstract quote  

Focal nodular hyperplasia (FNH) is a benign liver lesion incidentally discovered with increasing frequency because of the proliferation of imaging studies.

Radiographic characterization can diagnose this pathologic lesion and nonoperative therapy is the standard of care. However, if radiographic studies and fine needle biopsy are inconclusive, operative intervention may be required.

Depending on the anatomic location of the lesion, biopsy and/or resection can be performed laparoscopically. A patient with biliary dyskinesia and focal nodular hyperplasia is presented.

She underwent laparoscopic cholecystectomy with excision of the FNH. This paper reviews the case as well as the diagnosis and treatment of FNH.
Hepatic adenoma and focal nodular hyperplasia: diagnosis and criteria for treatment.

De Carlis L, Pirotta V, Rondinara GF, Sansalone CV, Colella G, Maione G, Slim AO, Rampoldi A, Cazzulani A, Belli L, Forti D.

Department of Surgery and Abdominal Transplantation, Niguarda Hospital, Milan, Italy.

Liver Transpl Surg. 1997 Mar;3(2):160-5. Abstract quote  

Focal nodular hyperplasia (FNH) and adenoma are rare benign hepatic tumors, and the standards for diagnosis and treatment still remain controversial. Usually adenoma is an indication for resection, due to its tendency to bleed and to degenerate; FNH, on the contrary, may be treated conservatively. Preoperation differential diagnosis is, however, difficult, often impossible.

MATERIALS AND METHODS: Thirty-eight patients with presumed hepatic adenoma and/or FNH were studied at our department from 1984 to 1996. Preoperative assessment included clinical evaluation and symptoms, laboratory tests, liver biopsy, ultrasound scan, computed tomography scan, magnetic resonance imaging, scintigraphy, and angiography. Thirteen patients had a presumed diagnosis of FNH, 16 of adenoma, and 9 of undetermined benign lesions; 27 had hepatic resections (3 with laparoscopic technique), and 11 were not operated on and are actually under a strict follow-up observation.

RESULTS: The final diagnosis was 19 FNH and 19 adenomas (2 of which contained areas of hepatocarcinoma). Presumed diagnosis was confirmed in 71% of cases. Use of oral contraceptives, abdominal symptoms, and pathologic liver test results were frequent in patients with adenomas. There were no deaths after surgery. All resected patients were tumor free during the follow-up, and in 10 of the 11 nonoperated cases, the size of the nodules remained unchanged. We conclude that precise diagnosis of these benign liver tumors remains difficult and sometimes impossible, despite new imaging techniques. Hepatic resections can be performed under very safe conditions; laparoscopic surgery may play a role in selected cases. Adenomas and uncertain cases are clear indications for surgery. Only when a diagnosis of FNH can be firmly confirmed in asymptomatic patients is strict observation without surgery recommended.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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