LIVER TRANSPLANTATION FOR HEPATIC MALIGNANCY

LIVER TRANSPLANTATION FOR HEPATIC MALIGNANCY

As a general rule, malignancy in a solid organ is not an indication for transplantation. This is because of the risk of recurrence with immunosuppression post transplant; immuno suppression suppresses not only the host’s immunity preventing rejection of the transplanted graft but also the host’s immunity against cancer, which may cause rapid pr ogression of disease after transplantation. However, LT is one area where a total hepatectomy in selected patients can remove the entire disease en bloc and give patients a full chance of cure. LT is widely Jacques Caroli , 1902–1979, Professor of Medicine, St Antoine Hospital, Paris, France, described the disease in 1958. Daniel Alagille , 1925–2005, Eminent Professor of Paediatric Hepatology , Hôpital Bicêtre, Paris, France, first described this condition in 1969. George Budd , 1808–1882, Professor of Medicine, King’s College Hospital, London, UK, described this syndrome in 1845. Hans Chiari , 1851–1916, Professor of Pathological Anatomy , Strasbourg, Germany (Strasbourg was returned to France after the end of the First World War, in 1918), gave his account of this condition in 1898. John Fielding Crigler , 1919–2018, American pediatrician, described the rare inherited disorder Crigler–Najjar syndrome in 1952. Victor Assad Najjar , 1914–2002, Lebanese–American pediatrician and microbiologist, described the rare inherited disorder Crigler–Najjar syndrome in 1952. /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF α /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF indicated as a curative treatment for selected patients with HCC, haemangioendothelioma and hepatoblastoma. Because of its association with CLD, up to 25% of LTs in the UK are in patients with HCC. Using the siz e and number of HCCs on pretransplant imaging, there are a number of criteria that - aim to select patients who have HCC with favourable tumour biology and hence a good outcome following LT . The two main staging criteria of HCC for the indication of LT are the Milan and the University of California San Francisco (UCSF) criteria. With recent advances in diagnostic modalities and chemo - therapeutic agents, LT has been carefully expanded to patients

Aetiology in children Drugs and toxins Hepatitis A and E Acute Wilson’s disease Biliary atresia -antitrypsin de /f_i ciency 1 Autoimmune hepatitis Sclerosing cholangitis Caroli’s syndrome Wilson’s disease Cystic /f_i brosis Progressive familial intrahepatic cholestasis Alagille’s syndrome Glycogen storage disease (types 3 and 4) Tyrosinaemia type 1 Budd–Chiari syndrome Any aetiology leading to hepatopulmonary syndrome or portopulmonary hypertension Crigler–Najjar syndrome Urea cycle defects Hypercholesterolaemia Organic acidaemias Glycogen storage disease type 1 Maple syrup urine disease Porphyria Unresectable hepatoblastoma (without active extrahepatic disease) Unresectable benign liver tumours with disabling symptoms

Evaluation of potential recipients for L T /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF with other primary or secondary malignancies of the liver. Cholangiocarcinoma (CCA) has been an uncommon indica tion for LT for nearly three decades. Recently there has been more interest with wider adaptation of the Mayo protocol, which involves strict patient selection, intensive pre-LT chemo radiation therapy , staging laparoscopy to assess tumour spread and then transplantation. Five-year survival has been reported in the range of 55–65% for hilar CCA in patients with primary sclerosing cholangitis (PSC), who get these cancers more com monly than de novo CCAs. Summary box 89.2 LT f o r h e p a t i c m a l i g n a n c y /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF The Mercedes-Benz sign takes its name from the insignia displayed on the bonnet of a Mercedes-Benz car.

Evaluation is undertaken by a multidisciplinary team, including a transplant surgeon and hepatologist Determine the presence of physical and mental health comorbidities Exclude malignancy and systemic sepsis Determine any contraindications Determine if the patient will bene /f_i t from LT with an acceptable quality of life Determine if the disease is suf /f_i ciently advanced to meet the minimal listing criteria for LT (e.g. UK end-stage liver disease [UKELD] score 49 or more) Determine the availability of family or social support and probable ability to cope psychologically with LT and comply with immunosuppression Optimise recipient condition before LT LT for HCC simultaneously treats the tumour and the underlying liver disease LT for HCC represents 15–50% of all transplants performed in most centres Milan criteria allow selection of HCC patients for LT, with improved overall and disease-free survival Milan criteria (one lesion ≤ 5 /uni00A0 cm, or three or fewer lesions ≤ 3 /uni00A0 cm each) UCSF criteria (one lesion ≤ 6.5 /uni00A0 cm, or three or fewer lesions ≤ 4.5 /uni00A0 cm each, with a total tumour diameter ≤ 8 /uni00A0 cm) UK HCC criteria (one lesion <5 /uni00A0 cm, or /f_i ve or fewer lesions all ≤ 3 /uni00A0 cm, or a single tumour >5 /uni00A0 cm and ≤ 7 /uni00A0 cm in diameter with no evidence of progression over a 6-month period) Tumour recurrence after LT for HCC ranges between 8% and 20% depending upon the criteria followed Primary malignant liver tumours constitute just over 1% of all childhood cancers The most common tumours that require LT in children are hepatoblastoma and HCC CCA, colorectal and neuroendocrine liver metastases are among the new indications for LT