Kishichiro Watanabe

Overlap of idiopathic portal hypertension and scleroderma: report of two autopsy cases and a review of literature.

Abstract Idiopathic portal hypertension (IPH) is characterized by dense fibrosis of portal tracts and portal venous obliteration. Little is known about the etiopathogenesis of IPH. Association of various autoimmune diseases such as systemic lupus erythematosus in IPH suggests that IPH may share immunological disturbances with such autoimmune diseases. We recently experienced two autopsy cases presenting with both diffuse scleroderma and IPH. Dense fibrosis was found in both the dermis and intrahepatic portal tract of these cases. In addition, small vascular damages were commonly observed to various degrees in these fibrotic areas of both organs. The activation of fibroblasts and vascular damages mediated by various growth factors and cytokines reportedly involved in the dermis in scleroderma might have also been operative in portal tracts in these two cases of IPH. A review of literature disclosed eight overlapping cases of IPH and scleroderma (middle‐ to old‐aged females), and scleroderma was diagnosed earlier than IPH. These findings suggest that similar pathogenetic processes are operative in the dermis as well as in the portal tracts of the liver in these cases.

Carcinoma of the thyroid at a young age—a review of 23 patients

Twenty‐three children and adolescents with thyroid carcinoma who were 20 years old or younger at the time of initial treatment were followed for a mean time of 9.5 years. Of the tumours, 21 were papillary and two follicular carcinomas. The majority (91%) of the 23 cases showed extra‐thyroidal extension of the tumour. An association with chronic thyroiditis was observed in 30% of the cases. There was no history of irradiation of the head and neck in any patients. Relapse occurred in eight patients, lung metastases in two and local recurrences in six; one patient died from lung metastases 3 months after initial treatment. Male patients, advanced stage of tumour, less differentiated histological features and DNA aneuploidy were associated with a higher frequency of relapse. The prognosis of thyroid carcinomas in children and adolescents is not so good as is generally believed.

Primary hepatic leiomyosarcoma in a woman after renal transplantation: Report of a case

In contrast to malignant lymphomas or skin cancer, smooth muscle tumors including leiomyosarcoma are rarely associated with transplant recipients. We herein present a 33-year-old woman with end-stage renal disease who received a transplant at 27 years of age. Four years after the transplantation, at age 31, she underwent a mastectomy because of primary right breast cancer, which was found to be a 5-mm-sized mucinous carcinoma with no regional lymph node metastasis. Six years after the transplantation, a liver tumor was unexpectedly discovered. An explorative laparotomy revealed a well-encapsulated tumor occupying the posterior portion of the right lobe of the liver. The patient underwent a posterior segmentectomy. Histologically, the tumor possessed intermingling fascicles of spindle cells with eosinophilic cytoplasm and elongated nuclei. Based on an immunohistochemical examination, the tumor cells were positive for the muscle-associated antibody. In addition, RNA probes for Epstein-Barr virus were negative based on in situ hybridization. The histologic, immunohistochemical findings were considered to be diagnostic for leiomyosarcoma, which is a low-grade malignancy. Two years after surgery, the patient is doing well with no recurrence of liver tumors or breast cancer.

Massive hemorrhage in a patient with intestinal Behçet's disease: report of a case.

Abstract Major gastrointestinal bleeding is a rare manifestation of intestinal Behçets disease. We report herein the case of a 64-year-old man with intestinal Behçets disease complicated by myelodysplastic syndrome who suffered massive hemorrhage. Colonoscopy demonstrated ulceration of the entire colon from the cecum to the rectum, characterized by punched-out ulcers. Angiography demonstrated apparent extravasation of contrast material in the terminal ileum, and embolization was not successful. Continued and massive bleeding necessitated surgical resection of the involved segment of ileum; however, massive bleeding recurred. Re-endoscopy showed oozing hemorrhage from the multiple colon ulcerations. Intra-arterial prednisolone injection therapy was given, following which the melena gradually subsided and completely stopped within a few days.

CORRELATION OF MORPHOLOGIC SUBTYPES OF LIVER CIRRHOSIS WITH EXCESS ALCOHOL INTAKE, HBV INFECTION, AGE AT DEATH, AND HEPATOCELLULAR CARCINOMA: A Study on 234 Autopsy Cases in Japan

Two hundred thirty‐four autopsy cases of liver cirrhosis were examined to correlate the tissue HBV markers and excess alcohol intake with the type of liver cirrhosis, and the incidence of hepatocellular carcinoma (HCC). The following four groups were classified as follows: (1) HBV marker‐positive alcoholic group A, (2) HBV marker‐negative alcoholic group B, (3) HBV marker‐positive non‐alcoholic group C, and (4) HBV marker‐negative nonalcoholic group D. Macronodular cirrhosis predominated in groups, A, C, and D, while in group B macronodular and micronodular cirrhosis were almost of the same frequency. The mean age at death of the patients with macronodular cirrhosis of HBV‐positive alcoholic group A was similar to that of HBV‐positive non‐alcoholic group C but lower than that of HBV‐negative alcoholic group B, suggesting a longer survival of alcoholics without HBV infection than that with HBV infection, when patients had macronodular cirrhosis at autopsy. In HBV‐negative alcoholic group B, patients with macronodular cirrhosis had a higher mean age than those with micronodular cirrhosis, while in HBV‐positive alcoholic group A, the mean age of patients with either cirrhosis was similar. This suggested that in the absence of HBV infection, macronodular cirrhosis in alcoholics may be related to the increased life span that allows a conversion of micronodular cirrhosis into macronodualr one, and in HBV‐positive alcoholics it may arise in relation to HBV infection. HCC was frequently associated with macronodular cirrhosis, regardless of the presence or absence of alcohol abuse or HBV infection, but rare in micronodular cirrhosis.

Pseudolipoma of Glisson???s Capsule: Report of Six Cases and Review of the Literature

We report six cases of pseudolipoma of Glissons capsule. The patients ranged from 53 to 83 years old (mean 67.7 years), and all were men. Pseudolipoma of Glissons capsule was detected by imaging studies before operation in two cases, and noted incidentally in the remaining four. Computed tomography demonstrated a subcapsular low-density nodule with or without a high-density spot. All but one patient had a history of abdominal operation. Grossly, the pseudolipoma was a solitary encapsulated nodule on the surface of the anterior aspect in five, and posteroinferior to the right lobe in one. The pseudolipomas ranged from 0.5 to 2 cm in diameter, were white to yellowish-white and elastic-firm. Histologically, the nodules had a dense fibrous capsule and contained necrotic mature fat tissue. In addition, three showed focal calcification. Blood vessels were rarely found within the capsule. These findings strongly suggest that history of abdominal operation, advanced age, sex, and poor blood supply to the nodule are causal to their development. Although only 18 cases have been reported previously, our six cases during but a 3-year period suggests that it may not be as rare as believed.

Autoimmune hepatitis associated with bile duct injury resembling chronic non-suppurative destructive cholangitis

Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) are representative autoimmune liver diseases in which hepatocytes and intrahepatic bile ducts, respectively, are selectively damaged by autoimmune mechanisms. Bile duct injury and loss is characteristic of PBC and chronic non‐suppurative destructive cholangitis (CNSDC), in particular, is a histological hallmark of PBC. In this report, we present an unusual case of AIH accompanied by CNSDC‐like bile duct injury in a 46‐year‐old woman. The patients serum aminotransferase level was abnormally high. The serum levels of alkaline phosphatase, γ‐GTP and IgG were also elevated, but the IgM level was within normal limits. The titer of antismooth muscle antibody (SMA) was 1:80 , while antinuclear autoantibody (ANA) and the M2 fraction of antimitochondrial antibody (AMA) were both negative. Liver biopsy disclosed CNSDC‐like bile duct injuries and severe interface hepatitis and lobular hepatitis with perivenular zonal necrosis were observed. The aggregate score of the International Autoimmune Hepatitis Group corresponded to the category of probable AIH. Immunohistochemically, histocompatibility leukocyte antigen‐DR, which is aberrantly expressed in the damaged bile ducts of PBC, was not found in the injured bile ducts of this case. Laboratory data were much improved by treatment with prednisone, but ursodeoxycholic acid was not effective. Although the possibility of an overlapping syndrome of AIH‐ and AMA‐negative PBC could not be excluded, this case was diagnosed as AIH with CNSDC‐like bile duct lesions.

Absence of Epstein-Barr virus (EBV) in intrahepatic cholangiocarcinoma confirmed by lack of EBV-coded nuclear RNA and latent membrane protein-1.

Studies are disclosing that Epstein–Barr virus (EBV) is involved in the aetiology of various neoplasms including undifferentiated carcinomas of the aerodigestive tract. The aetiology of intrahepatic cholangiocarcinoma (ICC), a malignant neoplasm arising from intrahepatic biliary epithelia, has yet to be fully evaluated. To date, two cases of EBV‐related ICC have been reported, and they presented foci of lymphoepitheliomatous undifferentiated carcinoma components.

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.

Hepatocellular prolapse of hepatic portal tracts and subendothelial space of central veins in idiopathic portal hypertension.

We report a case of idiopathic portal hypertension (IPH) with unusual liver pathology. The liver showed changes similar to these previously reported in IPH and, in addition, we observed the unusual features of prolapse of hepatocytes into portal tracts and also into the subendothelial space of hepatic veins. Hepatocyte prolapse into hepatic veins has previously been reported only in patients with a history of androgenic steroid therapy and immunosuppressive therapy. We speculate that, in our case, prolapse of hepatocytes could be related to the abnormal intrahepatic blood flow or to intrahepatic vasculopathy.