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Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61‐year‐old male with a two‐year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistocyto‐chemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32‐year‐old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed. ACTA PATHOL JPN 38: 883∼896, 1988.

Multiple serous cysts involving only the hepatic hilum and larger portal tracts were found incidentally in 8 autopsy cases with several underlying liver diseases. The cysts were mostly small in size, and did not communicate with the lumena of the biliary tree. The lining of the cysts consisted of a single layer of columnar or cuboidal epithelium and the surrounding fibrous tissue was scanty. These were not associated with polycystic disease of the kidneys or cystic dilatation of the biliary tree. Cysts were absent in the liver parenchyma and an association with von Meyenburg complexes was rare. In the vicinity of the cysts there were many lobules of the glands which are physiologically present in the periductal connective tissue of the large intrahepatic bile ducts. It was noted that some of these glandular elements around the cysts disclosed varying degrees of cystic luminal dilatations. Almost all patients with such cysts had severe portal hypertension and over a half had occluding thromboemboli in the portal veins. These results lead us to propose the hypothesis that multiple cysts involving the hepatic hilum and the larger portal tracts are derived from the periductal glands in close association with an intrahepatic circulatory disturbance of the portal venous system.

ABSTRACT— The morphology and distribution of the glandular elements around the intrahepatic bile ducts, hitherto poorly described, were examined in autopsied human livers with the aid of postmortem cholangiographs. The glands could be divided into intramural and extramural. The former were small in number, scattered within the bile duct walls, and were simple tubular mucous glands. The latter were more abundant, located in the periductal connective tissue, and were branched tubuloalveolar seromucous glands. Serial section observations revealed that neither gland communicated with the hepatic parenchyma, and the extramural glands drained into the large bile duct lumina via the conduits. The mucous cells of both glands contained neutral, carboxylated and sulfated glycoproteins. The extramural glands were distributed from the hepatic to the segment ducts in almost all livers, and were also discerned around the area ducts in two‐fifths of the livers. The glands seemed to decrease in number as the bile ducts became more branched.