a 33-year - old woman was admitted to our hospital with a two - month history of abdominal distension and edema . the patient had no prior history of drug usage , including oral contraceptives , and no relevant family medical history . upon admission , the patient underwent a physical examination , which revealed diffuse hepatomegaly , pitting edema in both legs , however no abnormal cardiac murmur . the laboratory findings indicated abnormalities including a hemoglobin level of 7.5 g / dl , a platelet count of 18,000/mm , as well as a slightly elevated serum alanine aminotransferase ( 50 u / i ) and aspartate aminotransferase ( 56 u / i ) . moreover , the hepatitis b virus antigen was negative , and the titer of the hepatitis b virus antibody and alpha - fetoprotein were normal . the clinical and radiological evaluation showed no evidence of rendu - osler - weber disease or hemangiomas in the extrahepatic region . an ultrasonography revealed a diffuse heterogeneous echoic infiltrative mass containing numerous and various - sized hypoechoic nodules involving the entire liver ( fig . 1a ) . a doppler ultrasonography revealed no remarkable tumor vascularities except for the underlying hepatic parenchymal vascular flow ( fig . a hepatic dynamic ct was performed and demonstrated the multifocal enhancing hepatic nodules on both the arterial and portal phases , as well as an increased area of contrast enhancement with a centripetal filling pattern of nearly the entire hepatic tumor on a delayed phase ct ( fig . however , there was no remarkable tumor calcification or intervening normal hepatic parenchyma in either of the hepatic lobes . subsequently , a 99mtc - labeled red blood cell scan was performed and the blood pool images were taken at five hours after inhomogeneous diffuse activity in the entire hepatic mass ( fig . 1 g ) . however , no extrahepatic mass with abnormally increased radioactivity was observed on this 99mtc - labeled red blood cell scan . our differential diagnoses included a diffuse hemangiomatosis , epithelioid hemangioendothelioma , and angiosarcoma . to rule out a hepatic angiosarcoma , an ultrasound - guided automated gun biopsy , using an 18-gauge core needle was performed in the right hepatic lobe . before the biopsy , the patient 's platelet count was 170,000/mm after infusion of fresh frozen plasma . the patient 's histology revealed prominent cavernous vascular proliferation and fibrosis without angiosarcomatous components ( fig . further , the patient showed no hemangiomas in other regions of the body , including the skin . the patient continued to complain about continuous abdominal distension , which rapidly progressed to the deterioration of hepatic function . it occurs in all age groups , although hepatic hemangiomatosis occurs predominantly in infants and may cause life threatening conditions ( 1 ) . however , diffuse hepatic hemangiomatosis in adults is very rarely encountered and moreso when observed without any other organ involvement ( 2 - 4 ) . although several cases of long - term adult survival of diffuse neonatal hemangiomatosis have been reported ( 5 , 6 ) , the etiology and natural history of diffuse hepatic hemangiomatosis remains unclear . the histological characteristics of hemangiomatosis includes the presence of large vascular channels in both the normal - appearing hepatic parenchyma and the cavernous tumor region ( 2 ) . the histological findings of our ultrasound - guided biopsy specimen revealed prominently increased vascular proliferation and fibrosis with no sarcomatous change on staining ( fig . 1h ) , as well as vascular endothelial cells showing positive staining on immunochemical stains , which included masson 's trichrome stain and the gomori reticulin stain . as no reliable clinical or radiological findings have been reported for the differential diagnosis of hepatic hemangiomatosis from rare primary vascular hepatic tumors such as hepatic hemangioendothelioma or angiosarcoma , the diagnosis relies solely on the histological findings ( 7 ) . also , because it is so rare , only a few reports document the imaging findings of diffuse hepatic hemangiomatosis . however , these imaging findings are similar to those of other common hepatic hemangiomas , including variable echoic lesions on the ultrasound , vascular pooling of contrast material within the tumor on angiography , a centripetal enhancement pattern on dynamic enhancement studies , and bright tumor signal intensities on t2-weighted mr images ( 2 , 3 , 8) . in our patient , the ultrasonography findings included a heterogeneous echoic infiltrative hepatic mass containing numerous hypoechoic nodules . compared to the histological findings our case showed a delayed centripetal contrast - enhancement pattern of the tumor on a dynamic ct and diffuse uptake on vascular pooling images from a 99mtc - labeled red blood cell scan . because our case revealed imaging findings similar to hepatic hemangiomas , epithelioid hemangioendotheliomas , and angiosarcomas , diffuse hepatic hemangiomatosis although previous reports have cited the role of steroid medications in the development of hepatic cavernous hemangiomas ( 10 ) and the administration of metoclopramide medications in a patient with diffuse hepatic hemangiomatosis ( 8) , no history of steroid or estrogen use was documented in the reported cases of diffuse hepatic hemangiomatosis , including our patient . the prognosis of diffuse hepatic hemangiomatosis without extrahepatic involvement is still unclear because it occurs so rarely and has variable clinical courses in affected patients . in the literature , various prognoses have been reported for diffuse hepatic hemangiomatosis without extrahepatic lesion in adults ( 2 , 3 , 8 , 11 ) . in our patient , the possibility exists that a minimal amount of the remaining normal hepatic parenchyma with extensive hepatic hemangiomatosis involving the entire liver may have caused hepatic failure and subsequent patient death . more cases of diffuse hepatic hemangiomatosis are needed to establish their natural course and prognosis . despite its rarity , we concluded that the diagnosis of diffuse hepatic hemangiomatosis in adults can be suggested in patients with a diffusely involved hepatic tumor showing radiologic findings of hepatic hemangioma .