A human cell line (KMCH-1) derived from a surgical specimen of combined hepatocellular and cholangiocarcinoma has been established. The original tumor consisted of both hepatocellular carcinoma of the trabecular type and cholangiocellular carcinoma. This cell line has been maintained for 26 months through 75 passages. KMCH-1 cells show characteristics of adenocarcinoma on light and electron microscopy. They proliferate in culture in a pave stone arrangement. The doubling time of these cells at the 24th passage was 39 hr. Chromosome analysis revealed a chromosome number ranging from 60 to 98, with a modal number of 74. KMCH-1 cells produced tumors several months after subcutaneous and intraperitoneal transplantations into athymic nude mice. Histologically, the subcutaneous tumors were poorly differentiated adrenocarcinoma, while intraperitoneal tumors were moderately to well-differentiated adenocarcinoma. Hepatocellular carcinoma components were not observed. Thus, KMCH-1 cells demonstrate the feature of cholangiocellular carcinoma in vitro and in vivo. This KMCH-1 cell line is the first established combined hepatocellular and cholangiocarcinoma cell line and may contribute to further investigation of combined hepatocellular and cholangiocarcinoma.

A new human hepatocellular carcinoma (HCC) cell line, KYN‐2, has been established from a surgical specimen obtained from a 52‐year‐old Japanese male HCC patient. The originally resected HCC was classified as pleomorphic HCC corresponding to Edmondson‐Steiner's grade III with a thick trabecular to solid arrangement. The cell line has been maintained for 17 months through 35 passages. Morphologically, the KYN‐2 cells have retained the characteristics of the original HCC, being pleomorphic and composed of various types such as cells with relatively small, polygonal, eosinophilic cytoplasm and oval‐shaped nuclei with a marked tendency to pile up, flat cells with abundant clear cytoplasm and oval‐shaped nuclei, and many multinucleated giant cells, proliferating in a pavement‐like cell arrangement. Some junctional complexes and a number of microvilli are evident between the cells by electron microscopy. Functionally, these cells were found to secrete albumin, α,‐acid glycoprotein, α1‐antitrypsin, ceruloplasmin, transferrin, complement C, fibrinogen, fibronectin, prothrombin, retinol‐binding protein (serum type), α‐fetoprotein (AFP), carcinoembryonic antigen (CEA), ferritin and β2‐microglobulin in chemically defined medium (CDM). The secretion of AFP and CEA is apparently dependent upon culture medium and passage. The doubling time of cells growing in serum‐containing medium at the 14 th passage was 84 h, and those of cells in serum‐containing medium, HB101 (serum‐free medium) and CDM at late passage were 28,68, and 42 h, respectively. Chromosome analysis revealed that the chromosome number ranged from 56 to 69 without a mode, and the presence of marker chromosomes. HB virus DNA sequence was not detected by hybridization analysis. The tumorigenicity of KYN‐2 cells was identified by development of tumors in nude mice after subcutaneous injection of the cells; the tumors showed an appearance basically similar to that of the original HCC. Thus, these findings suggest that the KYN‐2 cell line is available as a new human HCC cell line and should be useful for various studies on HCC. ACTA PATHOL JPN 38: 953‐966, 1988.

A new human extrahepatic bile duct carcinoma cell line (KMBC) was established from a serially transplanted tumor in nude mice that originated from a surgically resected tumor from a 73‐year‐old Japanese man; the cell line has been maintained for 5 five years. KMBC cells proliferate in a monolayered sheet with a population doubling time of 30 hours. Chromosome number was distributed in a range from 37 to 44, with modal numbers of 40 and 41. KMBC cells and the reconstituted tumor in a nude mouse showed moderately to poorly differentiated adenocarcinoma and possessed various functional characteristics of extrahepatic bile duct carcinoma. KMBC cells secreted carbohydrate antigen 19‐9, tissue polypeptide antigen, carcinoembryonic antigen, ferritin, β2‐microglobulin, fibronectin, and α2‐macroglobulin and produced glutamic oxaloacetic transaminase and alkaline phosphatase. KMBC is the second established cell line that originated from a human extrahepatic bile duct carcinoma in the world literature, and it will be applicable to various experiments.

Two cases of Cornelia de Lange syndrome associated with infantile haemangioendothelioma of the liver and Wilms' tumour are reported. The patients showed the characteristic facies of the Cornelia de Lange syndrome, with synophrys, long curly eyelashes and small upturned nose, and physical features, including generalized hirsutism, monodactyly, syndactyly and clinodactyly. Post-mortem examination revealed annular pancreas, patency of the foramen ovale, duodenal atresia and evidence of cytomegalic infection. The cases are reported to document a possible association between malformations and neoplasms in this syndrome.