A case of spontaneous innominate artery perforation with pericardial tamponade.

We describe a case of a spontaneously occurring innominate artery perforation which, rather than resulting in immediate death, developed a subacute course with findings suggestive of an expanding mediastinal mass. The cause of the spontaneous perforation appears to have been a combination of atherosclerosis, infection, and infiltration of the underlying structures with malignancy. This is a unique case of subacute spontaneous perforation of a great vessel.

Hepatocellular adenoma in glycogen storage disease type IV.

The development of hepatocellular adenoma has been recognized in association with glycogen storage disease type I and, less often, with glycogen storage disease type III, but, to our knowledge, it has not been reported in glycogen storage disease type IV. We had the opportunity to study an 11-month-old male infant who underwent orthotopic liver transplantation for cirrhosis that developed in the setting of glycogen storage disease type IV. A clinically unsuspected hepatocellular adenoma was present in the explanted liver. Glycogen storage disease type IV should be considered as a potential precursor to the development of hepatocellular adenoma. Recognition of this association is important, both in terms of the differential diagnosis of tumors that occur in this setting and also to anticipate potential complications of this benign neoplasm.

Epstein-Barr virus infection in liver transplantation patients: correlation of histopathology and semiquantitative Epstein-Barr virus-DNA recovery using polymerase chain reaction.

Epstein-Barr virus (EBV) infection may complicate orthotopic liver transplantation, and can lead to hepatitis with subsequent graft failure and to benign and malignant lymphoproliferative disorders. Early diagnosis allows for prevention or treatment of complications. Histopathologic features of EBV infection in the liver vary and may be difficult to recognize. To delineate the morphologic features that allow for recognition we studied 61 biopsy specimens from 37 patients, correlating the results of EBV-DNA demonstration after polymerase chain reaction with histopathology of formalin-fixed, hematoxylin-eosin-stained liver biopsy specimens. DNA was extracted from fresh liver biopsy samples, and polymerase chain reaction was carried out with EBV primers (capsid protein gp220) using standard techniques and 25-cycle amplification. Epstein-Barr virus-related sequences after polymerase chain reaction were detected by DNA blot assay. Histopathologic features were classified into three categories on the basis of the semiobjective determination of the number and distribution of immunoblasts and other immature lymphocytes in portal tracts and sinusoids: highly suggestive (three biopsy specimens), indeterminant (one biopsy specimen), and negative (57 biopsy specimens). Only the three highly suggestive biopsy specimens had high levels of EBV-DNA. We conclude that the histopathologic features of EBV infection after orthotopic liver transplantation can be relied on to establish the diagnosis.

The enigma of leiomyoblastoma: spectrum of a rare gastric tumor.

Leiomyoblastoma is a rare, smooth muscle tumor of the stomach occurring chiefly in the antrum in patients between the fifth and seventh decades. The clinical spectrum, as illustrated in eight patients described in this report, ranges from an incidental finding to a highly malignant, metastasizing neoplasm. Although the large majority of leiomyoblastomas are benign, malignancy has been reported to occur in up to 12% of cases. Resection of the tumor with an appropriate full thickness segment of gastric wall is mandatory.

Successful late Kasai portoenterostomy.

This article describes an infant with extrahepatic biliary atresia who underwent a successful hepatoportoenterostomy at age 132 days in spite of a delay in diagnosis. Contributing to the delay in diagnosis of this case were the lack of appreciation of alcoholic stools, the presence of a gallbladder and distal common bile duct on ultrasound examination, and insufficient biopsy material for interpretation. This case emphasizes the variability in presentation and potential difficulties in diagnosis and the need for intraoperative cholangiogram and exploratory laparotomy in such cases. An approach to the patient with extrahepatic biliary atresia who is diagnosed late is discussed. A successful outcome can be achieved for such patients.