Association of Porphyria Cutanea Tarda and Primary Liver Cancer: Report of Ten Cases

Abstract

Studies of the association between porphyria cutanea tarda (PCT) and primary liver cancer have produced conflicting reports. The incidence of this liver tumor was studied in 138 PCT patients (7.2%) and in 358 non‐porphyric cirrhotic patients (5.6%), and no statistically significant differences were found between them (p>0.5). Nevertheless the incidence of such association was significantly higher (chi square=10.91, p<0.001) in the PCT group (24.3%) than in the non‐porphyric cirrhotic group (6.7%) when only males with underlying liver cirrhosis were considered. These results suggest that the high incidence of liver tumor in PCT is not merely related to hepatic cirrhosis. The cause of such as association remains unclear. Primary liver cancer may reveal a prior deficiency in uroporphyrinogen‐decarboxylase, but the possibility that the enzyme abnormalities were caused by the liver carcinoma also exists.

Aetiological aspects on primary liver cancer with special regard to alcohol, organic solvents and acute intermittent porphyria - an epidemiological investigation

“…Of our 6 cases with PLC and PCT, 4 were alcoholics and 1 had haemochromatosis. In a series of 10 cases with PCT who developed PLC, all were alcoholics with liver cirrhosis (Solis et al, 1982).…”

Acute hepatic porphyria and hepatocellular carcinoma

“…Several studies have confirmed the 100 to 200-fold risk of HCC in porphyria cutanea tarda, which does not belong to the group of acute hepatic porphyrias (Berman & Braun, 1962;Kordac, 1972;Solis et al, 1982). Porphyria cutanea tarda is associated with gross liver abnormalities (Cortes et al, 1980), including cirrhosis in many patients which may be the morphological basis for HCC in this type of porphyria.…”

Is Porphyria cutanea tarda a Risk Factor in the Development of Hepatocellular Carcinoma?

“…In a review [25], 12 such cases were noted; a further example has since been described by Solis ct al. [11], These cases all had photosensitive skin lesions but in several instances the pattern of porphyrin ex cretion was not consistent with true PCT.…”

“…proposed that the risk of developing hepatoma in patients with cirrhosis and PCT is greater than the risk in those with cirrhosis alone [7,8], Until recently this possible association had received scant attention else where; apart from the Czechoslovak studies there are only 12 cases of hepatoma arising in patients with PCT in the world literature [9][10][11]. The present report describes a patient with long-standing PCT who subsequently died of liver cell carcinoma.…”

“…A better-designed survey was recently conducted by Solis et al [11]. They retrospectively examined the incidence of hepatoma in 138 PCT patients and 358 cirrhotic patients without porphyria.…”