CLINICAL, BIOCHEMICAL AND PATHOHISTOLOGICAL ASPECTS IN 12 PATIENTS WITH ACUTE PORPHYRIA AND HEPATOCELLULAR CANCER

Sardh E#, Harper P*, Andersson DEH#

#Department of Internal medicine, Stockholm Söder Hospital and *Porphyria Centre Sweden, Karolinska University Hospital Huddinge, Stockholm, Sweden. Eliane.Sardh@sos.ki.se

The aim of the study was to retrospectively study 10 women and 2 men with acute porphyria (10 AIP, 1 HCP, 1 VP) who had developed hepatocellular cancer (HCC).

The mean age for diagnosis of HCC was 64.8 (range 53-76). Eight patients were investigated for HCC because of reactivation of porphyria symptoms and/or increased urinary excretion of porphyrin precursors and porphyrins. In two of them the liver tumour was already palpable. The tumour was discovered on abdominal palpation in two patients and in two others by routine hepatic ultrasound examination. The patients were treated surgically (7), with chemotherapy (1), with radiotherapy (1), palliative (2) or with a combination of ablation, alcohol injection and chemotherapy (1). Six patients have died due to HCC. The survival time was 4.2 years; range 0.3-13. Alfa feto protein was positive in two of 10 investigated patients. At the present a re-investigation of liver biopsy material and screening for various cancer markers is in process.

Conclusion: It is of primary importance to detect HCC as early as possible, allowing surgical intervention. Carriers of acute porphyria above 50 years should be submitted for control at least yearly using ultrasound technology to detect incipient HCC. This method shows so far the highest sensitivity and is cost effective.