Liver cancer risk is increased in patients with porphyria cutanea tarda in comparison to matched control patients with chronic liver disease.
OBJECTIVE
Patients with porphyria and chronic liver disease could be at high risk of developing hepatocellular carcinoma. To define the incidence of primary liver cancer and identify variables associated with the risk of cancer in patients with porphyria cutanea tarda in comparison to control patients.


METHODS
Fifty-three patients with porphyria cutanea tarda were enrolled in a prospective study (median follow-up 72 +/- 54.1 months; range 12-216) and matched individually to a control case according to age (+/-5 years), sex, duration of follow up (+/- 5 years), severity of liver disease, and hepatitis C virus infection.


RESULTS
During follow-up hepatocellular carcinoma developed in 18 patients with porphyria and in four control patients. Incidence of primary liver cancer was 4.8 and 1.3 x 100 patients/year in the overall series of patients and of controls, respectively. The cumulative probability of being tumor free was significantly lower in porphyria cutanea tarda than in matched controls (75 vs 95%). Variables independently associated with the risk of liver cancer were the presence of porphyria and cirrhosis at enrollment (Odds ratios: 5.3, 95% CI 1.4-19.3 and 3.0, 95% CI 1.2-7.6, respectively).


CONCLUSIONS
Patients with porphyria are at higher risk of developing liver cancer than matched control patients.
Fracanzani
A L
AL
Dipartimento di Medicina Interna, Università di Milano, Ospedale Maggiore IRCCS, Milan, Italy.
Taioli
E
E
Sampietro
M
M
Fatta
E
E
Bertelli
C
C
Fiorelli
G
G
Fargion
S
S
D016022:Case-Control Studies; D002908:Chronic Disease; D005260:Female; D006801:Humans; D015994:Incidence; D008107:Liver Diseases / Q000150:complications*; D008113:Liver Neoplasms / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D017119:Porphyria Cutanea Tarda / Q000150:complications*; D015995:Prevalence; D018570:Risk Assessment