dr . alexandre batista liver hemangiomas are the most common benign liver tumors . diffuse hepatic hemangiomatosis ( dhh ) is a rare disorder , usually occurring in the neonate , characterized by extensive replacement of liver parenchyma with hemangiomatous lesions . it is frequently associated with a palpable abdominal mass and high - output heart failure , with high mortality rate in neonates . dhh in the adult , especially without extra - hepatic involvement , is extremely rare . hemangiomatosis differs from multiple or giant hemangiomas in that the boundary of the lesion is ill - defined , while in the latter , the liver parenchyma surrounding the lesion is compressed by a fibrous capsule forming the wall of the hemangioma . an asymptomatic 68-year - old man was referred to our hospital due to the presence of numerous nodular hepatic lesions accidentally detected on ultrasound . there was a history of heavy drinking ( alcohol intake around 70 - 90 g / d ) and no prior history of medication or steroid intake . physical examination upon admission revealed diffuse hepatomegaly , with no associated cardiac murmur or leg edema . ui / l ) . abdominal ultrasound ( us ) showed multiple ill - defined sub - centimeter hyperechoic hepatic nodules , predominately in the right lobe , with no posterior acoustic shadowing or significant through transmission and absence of high vascularity on color doppler [ figure 1 ] . 68-year - old male referred to our hospital due to multifocal hepatic lesions , subsequently diagnosed as diffuse hepatic hemangiomatosis . a ) ultrasound examination demonstrates multiple ill - defined yperechoic nodules dispersed throughout the hepatic parenchyma , mostly sub - centimeter and with right hemi - liver predominance . countless predominantly sub - centimeter nodular lesions with low t1-weighted signal intensity ( si ) and moderately high t2-weighted si were depicted . liver lesions showed moderately restricted diffusion , and post - contrast fat - suppressed t1-weighted images showed predominantly hypovascular features with retention of contrast in later phases . a us - guided biopsy using an automated gun with an 18-gauge needle was performed collecting three representative fragments . 68-year - old male referred to our hospital due to multifocal hepatic lesions , subsequently diagnosed as diffuse hepatic hemangiomatosis . a ) axial fatsaturated t2-weighted image shows countless small - sized moderately bright lesions ( arrows ) . b ) diffusion weighted image shows these lesions with high signal intensity ( b = 600 sec / mm ) ( arrows ) , which may be seen in liver hemangiomas secondary to t2 shine - through effect . axial c ) pre - contrast and ( d - f ) post - contrast fat - saturated t1-weighted images show arterial homogeneous enhancement ( arrows , d ) , some exhibiting portal phase peripheral nodular enhancement ( arrow , e ) and late centripetal filling ( arrows , f ) . pathology revealed endothelial - lined sinusoidal proliferation with erythrocyte content , consistent with hepatic hemangiomatosis [ figure 3 ] . definitive diagnosis of dhh was established based on a combination of imaging and pathological findings and clinical / imaging follow - up . two years following the diagnosis , the patient 's hepatic function and imaging findings remain stable , with no relevant associated symptoms . 68-year - old male referred to our hospital due to multifocal hepatic lesions , subsequently diagnosed as diffuse hepatic hemangiomatosis . ultrasound guided biopsy samples stained with hematoxylin and eosin a ) 4  10 , b ) 40  10 ; c ) 40  10 ) . reveal numerous dilated nonanastomotic vascular spaces , which were lined by flat endothelial cells and supported by fibrous tissue endothelium . chronic inflammatory infiltrates with peri - portal distribution are also present ( black outlined asterisk , c ) . imaging plays a vital role in the detection and characterization of multifocal liver lesions in adults . there are numerous causes including benign and malignant neoplasms , infectious lesions , and inflammatory conditions . the us evaluation showed multiple sub - centimeter , ill - defined , hyperechoic nodules dispersed throughout the liver parenchyma , predominantly in the right hemi - liver , with very little or no flow seen on color doppler . the differential diagnoses included metastatic disease , multifocal nodular fatty infiltration of the liver , bile duct hamartomas ( bh ) , or other less - frequent entities such as dhh or angiosarcoma . metastatic liver involvement usually occurs in a patient with a known extra - hepatic primary neoplasm . small hypervascular liver metastases ( e.g. from breast or neuroendocrine tumors ) may mimic small early uniform enhancing hemangiomas ; however , these lesions tend to fade or wash out , whereas hemangiomas are more likely to maintain the si or fade toward background isointensity . also , the majority of liver metastases shows heterogeneous or ring enhancement on the arterial phase images , confirming their definite diagnosis . multifocal nodular fatty deposition is an unusual pattern of liver steatosis that consists of multiple round or oval fat foci randomly distributed throughout the liver . fat deposits appear isointense or hyperintense to the liver on in - phase images , dropping si on out - of - phase images . fat accumulation does not show restriction or shine - through effect on diffusion - weighted images . other clues indicative of multifocal fat deposition are the lack of mass effect , stability in size over time , and contrast enhancement similar to or less than that of the surrounding liver parenchyma . bh , also called von meyenburg complexes , originate from embryonic bile ducts that fail to involute . on ultrasound , bh have been described as innumerable tiny hypoechoic or hyperechoic foci measuring less than 10 mm and distributed uniformly throughout the liver , with some displaying a comet tail artifact . differences in echogenicity may be due to the size of the dilated bile duct component , which , at a certain size , would behave like other microcystic structures . on mri , bile duct hamartomas usually appear as multiple tiny cystic lesions , strongly hyperintense on t2-weighted images . after gadolinium administration , some authors described a thin rim of enhancement , which correlates with the compressed liver parenchyma surrounding the lesions . internal progression or retention it is very aggressive and sometimes may mimic hemangioma on dynamic studies , showing high si on t2-weighted images , since both tumors contain abundant blood - filled vascular spaces ; also , heterogeneous centripetal and progressive enhancement has been described . dhh is a rare , although benign , condition that is characterized by diffuse replacement of liver parenchyma by hemangiomatous lesions . the natural history and prognosis of hepatic hemangiomatosis are poorly understood due to its rarity . hemangiomatosis is different from multiple or giant hemangiomas in that the boundary of the lesion is ill - defined , while in the latter , the liver parenchyma surrounding the lesion is compressed by a fibrous capsule that forms the wall of the hemangioma . dhh can occur in all age groups , but is most frequent in the neonate where it can result in high - output cardiac failure and significant mortality . it is usually asymptomatic in adulthood , being extremely rare when not associated with giant cavernous hemangiomas and even rarer when no other organ is affected besides the liver . two patterns of dhh are described , the diffuse ( non - nodular ) and the nodular pattern , the former being the predominant pattern , representing approximately two - thirds of the cases . sonographically , the liver affected by dhh appears as homogeneous hyperechoic areas with poorly defined margins ( diffuse pattern ) or a heterogeneous echo pattern with multiple discrete or coalescent small hyperechoic nodules ( multinodular pattern ) . these nodular lesions are variable in size ( usually < 5 - 10 mm ) , appearing as countless oval or round hyperechoic nodular lesions . very little or no flow , the areas affected by hemangiomatosis may also display a diffuse non - nodular pattern composed of tiny cystic spaces separated by numerous thin hypointense septa , creating a lattice - like appearance or a diffuse nodular pattern consisting of multiple small discrete and coalescent nodules on t2-weighted imaging . the extremely low velocity of blood flow within hemangioma relates to the absence of flow void seen in mri imaging . dynamic contrast - enhanced mri shows heterogeneous enhancement in these areas during the arterial phase that becomes more homogeneous during portal and delayed phase imaging . the multinodular type , as was our case , exhibits small discrete and coalescent nodules with early homogeneous enhancement or the typical peripheral discontinuous enhancement during the arterial phase , showing uniform late retention of contrast . in conclusion , dhh with no associated cavernous hemangiomas and without extra - hepatic involvement is an extremely rare condition . despite its rarity , it can be suggested in adults presenting with multiple , predominantly sub - centimeter hepatic lesions demonstrating mri features of hemangiomas . mr imaging may be superior to us and computed tomography ( ct ) for the diagnosis and is the preferable cross - sectional imaging technique for long - term follow - up .
we present a case of a pathologically proven multinodular diffuse hepatic hemangiomatosis ( dhh ) with no extra - hepatic involvement in a 68-year - old male . 
 cavernous hemangioma is the most common hepatic tumor . 
 however , dhh , which is characterized by extensive replacement of liver parenchyma with hemangiomatous lesions , has been rarely reported in adults . 
 the etiology and clinical course are not completely understood because of its rareness , although the diagnosis might be suggested by the magnetic resonance imaging findings .