Interruption of aortic arch and hypoplastic left heart syndrome.

Interruption of the aortic arch and hypoplastic left heart syndrome in the same patient is exceptional. In the combined collections of the Registry of Cardiovascular Disease (St. Paul, Minnesota, U.S.A.) and the Registry of Congenital Heart Defects of the Rijksuniversiteit Gent (Gent, Belgium) three specimens were found with this unusual combination. These cases are herein described and compared with four similar cases previously reported in the literature.

Combined leiomyomatosis of the small intestine and colon.

We report a case of leiomyomatosis that involved the small intestine and colon. On gross examination, the bowel wall was irregularly thickened at various levels. Histologically, the lesions were characterized by multifocal, diffuse tumor masses that were present in the muscular layers. These masses consisted of proliferating smooth-muscle cells that often surrounded prominent blood vessels. Cytologic atypia was not observed, and the mitotic count was low, illustrating the benign nature of the tumors. Findings from immunohistochemical analysis of the lesions were essentially negative; vimentin, cytokeratin, actin, desmin, and S100 stains showed no reactivity in the tumor cells, despite positive internal controls. By reviewing the literature, we found several reports on related lesions that occurred in the gastrointestinal tract; diffuse leiomyomatosis of the esophagus and the colon have been reported. We did not find any report on the small intestinal variant of the disease; therefore, it seemed useful to term this new localization as leiomyomatosis of the small intestine and colon.

Adamantinoma of the tibia with pulmonary metastases and hypercalcemia.

Adamantinomas of long bones are rare primary malignant bone tumors. A case of a woman who died of pulmonary metastases of an adamantinoma of the tibia is presented. A unique feature of this case is the association with hypercalcemia. The association of hypercalcemia, hypophosphatemia, decreased parathyroid hormone levels and increased urinary cAMP excretion suggests a humorally mediated hypercalcemia. Histologic and ultrastructural analysis of the pulmonary metastases demonstrated that the tumor was composed of a heterogeneous cell population with mesenchymal and epithelial differentiation.

The Budd-Chiari syndrome caused by a zygomycete. A new pathogenesis of hepatic vein thrombosis.

The clinical history of a 60-year-old woman suffering from chronic lymphocytic leukemia with sudden deterioration and death is reported. The postmortem macroscopic and microscopic findings included pulmonary aspergillosis and pulmonary zygomycosis (mucormycosis). Hematogenous dissemination of the zygomycete causing cardiac zygomycosis, cerebral infarcts due to vascular occlusion by hyphae, and thrombosis of the major hepatic veins (Budd-Chiari syndrome) with submassive necrosis of the right liver lobe were also observed. To our knowledge, this is the second report dealing with occlusion of the hepatic veins caused by a fungus and the first study reporting the Budd-Chiari syndrome due to a mold of the subclass Zygomycetes.