INTRODUCTION: To elucidate the clinical presentation, diagnosis and management of primary hepatic carcinoid tumours, a literature search was conducted and summarized. MATERIALS AND METHODS: Published primary hepatic carcinoid tumour case reports and series were searched and selected in the Medline, EMBASE and Cochrane Library databases. RESULTS: Sixty-nine cases meeting the inclusion criteria were identified. Twenty-eight patients were male (28/69 = 40.6%). The median age at diagnosis was 50 years (range 8-83 years). The most common presentation described was abdominal pain (23/69 = 33.3%), or no symptoms at all (16/69 = 23.2%). Symptoms of carcinoid syndrome were described in 18.9% of cases (13/69). The most frequently secreted hormones were gastrin (7/69 = 10.1%) and chromogranin A. In 31.9% of patients (22/69), surgical treatment was not adopted. Of those treated surgically, 63.8% underwent a hepatic resection (44/69) and 4.3% a liver transplantation (3/69). After a median follow-up of 31 months (range 0-180 months), 39.1% of patients (27/69) died and 52.2% (36/69) survived. CONCLUSIONS: Primary hepatic carcinoids are an important entity in which the exclusion of different primary locations is necessary. When feasible, hepatic resection is the treatment of choice. Liver transplantation has been described in a small number of unresectable cases.
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