A case history of a 16-year-old boy with hepatocellular carcinoma and an intermediate deficiency of alpha1-antitrypsin (MZ phenotype) is presented. Previous reports have suggested that hepatocellular carcinoma may be associated with the Z variant of antitrypsin and either a severe or intermediate antitrypsin deficiency. The present case is unusual because of the rather high level of the serum trypsin inhibitory capacity for an MZ heterozygote (1.633 units), which may be due to involvement of the liver by the tumor or to a recent partial hepatectomy. PAS-positive antitrypsin globules were seen in the primary tumor and in nodules metastatic to the mesentery, as well as in nonneoplastic portions of the liver. Hepatocellular carcinoma is another disease state that may occur preferentially in individuals with either severe or intermediate deficiencies of alpha1-antitrypsin.
A clinicopathologic analysis of 15 patients with glassy cell carcinoma confirmed that this is a rare disease, having an incidence of 1.2%. It is a rapidly progressive and biologically aggressive disease with early extrapelvic metastasis. The five‐year survival and the median survival in our series were 28% and 14 months, respectively. The majority of patients (87%) were understaged, which may have played a role in the poor prognosis. Four of 12 patients (33%) were initially diagnosed as having a benign disease (false‐negatives). Defining of cytologic characteristics and differential features of this tumor may facilitate an early and more accurate diagnosis to improve prognosis. Cancer 52:307‐312, 1983.
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