Nobutatsu Takayanagi

Immunohistochemical study of hepatic angiomyolipoma

An immunohistochemical study was performed on nine hepatic anglomyolipomas (AML) found in eight patients. Histologically, the tumors were fundamentally composed of the three heterogeneous tissue components of blood vessels, smooth muscle cells (SMC), and fat cells, although the proportions and distributions were quite variable from tumor to tumor and from area to area in the same tumor. Additionally, cellular pleomorphism and atypia with occasional bizarre giant cells were found in the SMC component. This histologic feature might lead to a mistaken diagnosis of malignant neoplasm, and pathologists should therefore be aware of the broad histologic spectrum of hepatic AML. However, the Immunostalning patterns were basically the same in all nine tumors. All tumor components were negative for epithelial membrane antigen (EMA) and for cytokeratin. The spindle‐shaped SMC component of the tumor was occasionally positive for vimentin, desmin and alpha‐smooth muscle actin, whereas epithelioid SMC were negative for all three. Both the epithelioid and spindle‐shaped SMC were occasionally positive for S‐100 and neuron‐specific enolase. All types of SMC in the tumor, whether spindle, epithelioid, intermediate or pleomorphic SMC, were strongly positive for HMB‐45, a melanoma‐specific monoclonal antibody. Fat cells were occasionally positive for S‐100. Endothelial cells were positive for factor Vlll‐associated antigen. Among hepatic tumors HMB‐45 reactivity is, so far as we know, found exclusively in the SMC of AML, and the HMB‐45 reactivity of a hepatic tumor is thus clearly an important piece of information in the diagnosis of AML

Multiple angiomyolipoma of the liver.

Angiomyolipoma (AML) of the liver is extremely rare; we believe only about 60 cases, all solitary, have been reported. We present here the first reported case, to our knowledge, of multiple AMLs in the liver. Two masses were found in the liver of a 43-year-old Japanese woman complaining of abdominal discomfort by ultrasonography and computed tomography. With a preoperative diagnosis of hepatocellular carcinoma, a tumor in the right lobe 4.0 cm in largest diameter and one in the left lobe measuring 2.4 cm were resected. Histologically, both tumors were composed of smooth-muscle cells, fat cells, and blood vessels, and contained foci of extramedullary hematopoiesis. Although the smooth-muscle cell component was predominant and both tumors exhibited some pleomorphism, no mitosis was found. These histologic findings are consistent with the diagnosis of AML.

CT characteristics of intrahepatic, periportal, extramedullary hematopoiesis.

A case of primary myelofibrosis that showed intrahepatic periportal extramedullary hematopoiesis on CT and sonography is presented. Pathological correlation of the autopsy material was obtained. It should be included in the differential diagnosis of periportal abnormality on CT.

STUDIES ON THE PATHOGENESIS OF HEPATOCELLULAR CARCINOMA IN HBV-NEGATIVE ALCOHOLIC CIRRHOTICS

Ninety five cases of HBV marker‐negative cirrhosis with excess alcohol intake were examined clinicopathologically to obtain some clues and insights into the pathogenesis of hepatocellular carcinoma (HCC). The following data were obtained: (1) cases were divided morphologically into 37 cases of macronodular cirrhosis (MacCir), 16 mixed cirrhosis (MixCir), and 42 mi‐cronodular cirrhosis (MicCir), (2) the mean age at death was the oldest in MacCir (61 yrs), the youngest in MicCir (51 yrs), and intermediate in MixCir (59 yrs), (3) association of HCC was common both in MacCir and MixCir (78 and 63%, respectively) but infrequent in MicCir (17%), (4) all livers of MicCir with HCC had broad collapse and a small number of macronodules in noncancerous areas and the mean age of them was older than that of MicCir without both the collapse and macronodules (56 vs 48 yrs), (5) in total cases, the mean age at death of patients with HCC was 7 years older than that without HCC (60 vs 53 yrs), (6) the mean liver weight was the largest in MicCir (1,211 g), the smallest in MacCir (829 g), and intermediate in MixCir (1,022 g), (7) the incidence of MacCir was significantly higher in patients who had given up alcohol for more than one year before death than those without abstinence, and (8) neither the subtypes of cirrhosis nor the incidence of HCC was significantly related to the total amount of alcohol intake. These data indicate that the development of HCC in HBV‐negative alcoholics with cirrhosis occurs in relation to the development of macronodules and loss of liver weight, most likely along with the prolongation of the life span. ACTA PATHOL. JPN. 36: 1297–1305, 1986.

Calcification in caval membrane causing primary Budd-Chiari syndrome: CT demonstration.

We report two cases of membranous obstruction of the inferior vena cava (MOIVC) in which we observed patchy calcification in the membrane. The location of the calcification in the membrane was confirmed histologically. This finding was found histologically in four of eight cases of MOIVC, and it is a useful finding in the CT diagnosis of MOIVC.