Brachial artery aneurysm in an infant: case report and review of the literature.

A case is reported of a congenital aneurysm of the brachial artery in a 9-month-old boy. The lesion was excised and the artery reconstructed with an end-to-end anastomosis. Review of the English language literature showed that brachial artery aneurysms in children usually occur in association with arteritis, connective tissue disorder, or a syndrome of multiple aneurysms of unknown origin.

Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases.

The clinical, morphologic, and follow-up findings in 32 patients with a hitherto rarely reported tumor of the liver are reported. The study comprised 20 women (62.5 per cent) and 12 men (37.5 per cent) ranging in age from 19 to 86 years (average, 49.65 years). The tumors were discovered incidentally in four patients (12.5 per cent). Four patients (12.5 per cent) had jaundice attributable to the tumors; one of these patients experienced liver failure. One patient presented with an acutely painful abdomen due to hemoperitoneum. The remaining patients had nonspecific complaints. Grossly, the tumors were often multiple and involved both lobes of the liver. They were generally white and firm to hard. Microscopically, the neoplastic cells infiltrated sinusoids and intrahepatic veins of all sizes. Two types of tumor cells were identified--dendritic and epithelioid. Tumor cells were also vasoformative and synthesized Factor VIII-related antigen. Nine patients survived five years or longer. Two of these patients were alive five years, two nine years, one 12 years, and one 15 years after the onset of disease. Three patients died seven, ten, and 28 years, respectively, after the initial diagnosis. This vascular tumor has distinctive morphologic features that allow differentiation from sclerosing carcinoma and angiosarcoma. Although the prognosis is much more favorable than that for angiosarcoma, extrahepatic metastases occurred in nine of the patients (28 per cent) in this series. The biologic behavior of the tumor may be related in part to its matrix, which may show inflammation, dense sclerosis, and calcification.

Small nonpolypoid carcinomas of the large intestine.

Two small carcinomas of the colon were examined, one an intramucosal carcinoma and the other a small carcinoma invading the submucosa. Serial sections did not reveal any adenomatous hyperplasia or polyps in the vicinity. The colonoscopist must be aware of the potential clinical importance of any small mucosal excrescence. Although several studies have indicated that most colonic carcinomas develop from an adenomatous polyp, some carcinomas apparently arise de novo. Further study of the epidemiologic, etiologic, and prognostic factors relating to these apparently uncommon lesions is indicated.

Phenotypic characterization of hepatic proliferation. Antigenic expression by proliferating epithelial cells in fetal liver, massive hepatic necrosis, and nodular transformation of the liver.

Different forms of hepatocellular proliferation are seen in fetal livers, massive hepatic necrosis, and nodular transformation (nodular regenerative hyperplasia) of the liver. In an attempt to characterize the proliferating cells in these conditions, we studied the expression of several antigens by immunohistochemical methods. Alpha-fetoprotein (AFP), alpha 1-antitrypsin (AAT), a hepatocellular export protein, carcinoembryonic antigen (CEA), a marker of bile duct epithelial cells, and hepatitis B virus antigens (HBsAg, HBcAg), were localized by the peroxidase-antiperoxidase method in 11 fetal livers, 10 cases of nodular transformation, and 7 cases of massive hepatic necrosis. AFP was the most prevalent antigen in fetal hepatocytes. Many hyperplastic hepatocytes in nodular transformation contained AAT, but not oncofetal antigens, supporting the differentiated hepatocellular nature of these cells. A similar staining pattern was seen in two-cell-thick plates of hepatocytes in massive hepatic necrosis. In contrast, the ductlike structures at the periphery of necrotic lobules contained both AAT and CEA, suggesting that these cells exhibit features of hepatocytes and bile duct epithelial cells. Therefore, the appropriate term for these regenerating cells appears to be "ductular" or "biliary hepatocytes".

Gaucher disease: hepatic abnormalities in 25 patients.

Twenty-five patients with enzymatically confirmed Gaucher disease were selected for study of hepatic complications of the disorder. Hepatomegaly was noted clinically and confirmed by radioisotopic scan in all but 1 patient. The majority had abnormalities of serum transaminase, alkaline phosphatase, and sulfobromophthalein (BSP) clearance. Three patients had evidence of portal hypertension. Light microscopic examination of liver showed some variation in the pattern of the disease. Gaucher cells were present in all specimens. In 5 patients the distribution was in scattered foci throughout the liver lobule. In 14 patients there was prominent centrilobular accumulation of Gaucher cells. Extensive replacement of liver by storage cells and cirrhosis was documented in 3 patients. No patient was found to have amyloid deposits. The severity of hepatic involvement correlated with the occurrence of other severe complications of the disorder. The wide range of liver abnormalities in Gaucher disease should be considered in evaluating patients for participation in experimental therapeutic trials.

Hepatic injury associated with paraquat toxicity in humans.

Thirteen patients are reported who developed evidence of hepatic damage after exposure to paraquat and subsequently died. At autopsy, the main changes involved the bile excretory pathways. Ten of the thirteen cases had cholestasis, usually localized to the centrilobular zone. There was cholangiocellular injury involving the small and medium-sized bile ducts in portal areas. It consisted of shrinkage of cells, poor definition of outline, separation from the basement membrane, desquamation of cells into the lumen, infiltration of the wall by neutrophils and possible loss of integrity of the basement membrane. These bile duct lesions have not been previously described in association with paraquat toxicity. On the basis of the overall histologic findings in this study and extrapolation from experimental studies, it is hypothesized that paraquat injury to the liver is biphasic; it is initially hepatocellular but becomes cholangiocellular after the first 2 days.

B-scan echo-amplitude measurement in patients with diffuse infiltrative liver disease.

B-scan echo-amplitude measurements of the liver were recorded in a prospective clinical trial involving 56 persons--28 normal volunteers and 28 patients with Gaucher disease. The nonspecular liver parenchymal echoes were found to be abnormally elevated in Gaucher patients despite relatively normal liver biochemical values and absence of symptomatic liver disease. Echo amplitude corresponded directly to the amount and histologic distribution of abnormal glycolipid accumulation within the liver. The highest abnormal values were recorded when there was clumping of the glycolipid cells, producing discontinuities within the range of ultrasound resolution. B-scan echo-amplitude measurements accurately represent the degree of histologic abnormality in Gaucher patients, suggesting that this technique should be useful for monitoring enzyme replacement therapy.

Benign hepatocellular tumors: a regional survey.

A survey of benign hepatocellular tumors in a metropolitan area included six cases of hepatocellular adenoma, eight of focal nodular hyperplasia, and two of nodular transformation. Two thirds of the adenomas occurred in women using oral contraceptives; two had life-threatening complications. The focal nodular hyperplasias were generally asymptomatic tumors occurring in patients over a wide age range and apparently not related to oral contraceptive use. The two cases of nodular transformation were incidental findings in patients having abdominal surgery. One of them had used oral contraceptives.

LIver abnormalities in patients with Gaucher's disease.

Clinical and liver histopathological observations are described in 25 patients with Gaucher's disease. Although 24 patients had hepatomegaly, and the majority had abnormalities of serum transaminases, alkaline phosphatase, and BSP clearance, only 3 had evidence of portal hypertension and complications of advanced liver disease. Liver biopsies showed scattered foci of Gaucher cells in 5 patients and prominent centrilobular accumulation of Gaucher cells in 14 patients. Three patients had cirrhosis, which was associated with extensive replacement of the liver by storage cells. The severity of liver abnormalities correlated with the occurrence of other severe complications of Gaucher's disease. The wide spectrum of liver abnormalities in Gaucher's disease should be considered in evaluating trials of therapeutic enzyme replacement.

Nodular transformation (nodular "regenerative" hyperplasia) of the liver. A clinicopathologic study of 30 cases.

Nodular transformation, a rare hyperplastic condition of the liver, has been reported in patients with rheumatoid arthritis, Felty's syndrome, the CRST syndrome, and myeloproliferative disorders. Associated disorders in the present clinicopathologic study of 30 cases included the foregoing as well as extrahepatic neoplasms, endocrine disorders, and other diseases producing immune dysfunction; some patients had received drug therapy for a prolonged time. Clinically nodular transformation may be confused with cirrhosis; histologic evaluation of liver biopsy material is essential for diagnosis. Complications include portal hypertension, hepatic failure, and rupture of the liver. Histologic and experimental evidence suggests that nodular transformation is preneoplastic, possibly giving rise to hepatocellular adenomas or carcinomas. Experimental and clinical data suggest that drugs should be considered as possible etiologic factors in the development of the nodules.

Ground-glass cells in hepatocellular carcinoma.

Hematoxylin and eosin-stained sections representing ten cases of hepatocellular carcinoma showed many tumor cells with ground-glass cytoplasm identical to that found in hepatocytes containing hepatitis B surface antigen (HBsAg). However, the aldehyde fuchsin stain was negative, as were the were the immunoperoxisidase stains for HBsAg and core antigen (HBcAg). Electron microscopically, the ground-glass appearance corresponded to the presence of non-membrane-bound amorphous or fibrillar inclusions. Immunohistochemically, the ground-glass material reacted with antiserum to human fibrinogen, suggesting synthesis of this protein by the carcinoma cells. Although the ground-glass appearance in hepatocellular carcinomas may sometimes be associated with HBsAg, special stains or technics are necessary to confirm its presence.

Liver injury with alcoholic hyaline after intestinal resection.

We describe a patient who developed liver injury with alcoholic hyaline after intestinal resection. Malnutrition appeared to be a dominant factor in the origin of his liver injury since no hepatotoxic drug, alcohol, or toxic bacterial product from a blind loop could be implicated.

Histology of the liver in Wilson's disease: a study of 34 cases.

Biopsy or autopsy samples of liver from 34 patients with clinical evidence of Wilson's disease were studied histologically. Seven had early changes ranging from slight pleomorphism of hepatocytes to fatty metamorphosis, vacuolated nuclei, and focal necrosis. Seven other specimens from cases classified as chronic active hepatitis were characterized by periportal degeneration and necrosis, in addition to many of the changes of the first group. Periportal copper accumulation was found in three of these specimens and was considered helpful in the distinction from other forms of chronic active hepatitis. Specimens from four of the precirrhotic cases showed periportal atypical lipofuscin granules. The 20 cirrhotic specimens were macronodular or mixed micronodular-macronodular and showed a wide variety of patterns and cytologic changes. Eight contained Mallory bodies. Copper was demonstrated in 13 of 15 specimens with adequate copper stains. Some of the findings described here may suggest or confirm a diagnosis of Wilson's disease, but none is considered specific. A proper approach to the diagnosis should correlate the clinical, laboratory, and morphologic findings.

Anabolic steroid therapy and intrahepatic cholangiocarcinoma.

A 47-year-old man who had been treated with an anabolic steroid for refractory anemia developed an intrahepatic cholangiocarcinoma with metastases to abdominal lymph nodes and lungs. Microscopically, the tumor showed mucin production and was devoid of hepatocellular elements. Previous reports have suggested a possible relationship between anabolic steroid therapy and hepatocellular carcinoma. In many such cases, there are doubts about the histological diagnosis, malignant potential, and the nature of the association between the steroids and the tumors. The presence of distant metastases attests to the malignant nature of the tumor in the present case. Despite the apparent temporal eligibility of the steroid as an etiologic agent, a causative relationship between therapy and tumor is not established.

Myoepithelioma of minor salivary gland origin. Light and electron microscopical study.

A gingival tumor that invaded the anterior maxilla was removed from a 14-year-old boy and studied by light and electron microscopy. The tumor was composed exclusively of myoepithelial cells and appeared to be malignant. By light microscopy, the tumor appeared to be a poorly differentiated epithelial neoplasm of undetermined origin; however, electron microscopical examination showed myoepithelial differentiation, indicative of a salivary gland origin. To our knowledge, the present case represents the only confirmed myoepithelioma that shows features indicative of malignant potential. Myoepitheliomas may be related to mixed tumors of salivary glands.