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Oligomeganephronia (OMN) is a rare, renal hypoplasia, consisting of reduced number of hypertrophied nephrons. This disorder has been considered to be a congenital but not a genetic disease. We describe the first report, to the best of our knowledge, of familial cases of OMN; two male siblings ran rapidly downhill courses and died 11 and 8 days after births, respectively. In addition, the two patients had similar multiple anomalies; microcephaly, prominent glabella, hypertelorism, antimongoloid slant, epicanthal folds, broad nose, cleft lip and palate, downturned mouth, short philtrum, micrognathia, low set ears, hypospadias, and cryptorchism. Although the patients and the parents had normal G‐banded karyotypes, 4p monosomy syndrome is suggested from clinical features. The implications of this are discussed briefly. ACTA PATHOL. JPN. 35: 449–457, 1985.

An autopsy case of primary hepatic fibrosarcoma with severe hypoglycemia is reported. Only two cases have been previously reported. Histological features were well differentiated and partly indistinguishable from those of fibrous mesothelioma. ACTA PATHOL. JPN. 33 : 177-182, 1983.

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