High risk of primary liver cancer in a cohort of 179 patients with Acute Hepatic Porphyria.
OBJECTIVE
Previous studies have indicated a high risk of hepatocellular carcinoma in acute hepatic porphyrias. In this retrospective study we present the incidence of primary liver cancer and clinical characteristics in a cohort of 179 acute porphyria patients above the age of 50 years.


METHODS
Twenty-three cases with primary liver cancer were found either by a surveillance program or due to clinical suspicion. Standardized rate ratio was used to estimate the relative risk of primary liver cancer after indirect standardization. Survival data were calculated using the Kaplan-Meier method.


RESULTS
The mean age at diagnosis was 69 years. Hepatocellular carcinoma was found in 19 patients while four patients had cholangiocarcinoma or a combination of the two. Four patients had underlying cirrhosis. Mean tumour size was 4.3 cm in the surveillance group and 10.3 cm in the non-surveillance group (p = 0.01). The overall relative risk of primary liver cancer was 86 above the age of 50: 150 for women and 37 for men. Mean survival time was 5.7 years.


CONCLUSIONS
Acute hepatic porphyria carries a high risk of primary liver cancer above the age of 50 which warrants ultrasound surveillance. Sex distribution and frequency of cirrhosis differs from more common aetiologies of primary liver cancer.
Department of Internal Medicine, Karolinska Institutet, Stockholm South Hospital, 11883, Stockholm, Sweden, eliane.sardh@ki.se.
Eliane
E
Sardh
Staffan
S
Wahlin
Mikael
M
Björnstedt
Pauline
P
Harper
Dan E H
DE
Andersson
D000368:Aged; D000369:Aged, 80 and over; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology / Q000209:etiology*; D015331:Cohort Studies; D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D000623:Porphobilinogen Synthase / Q000172:deficiency*; D017094:Porphyrias, Hepatic / Q000150:complications* / Q000453:epidemiology; D012307:Risk Factors; D016019:Survival Analysis; D013548:Sweden / Q000453:epidemiology
D000623:Porphobilinogen Synthase