An Unusual Location of Neuroendocrine Tumour: Primary Hepatic Origin.

Although neuroendocrine tumours (NETs) of primary hepatic origin are extremely rare, most of NETs present with liver metastasis. When a NET is found in the liver, it must be treated to exclude metastasis from extrahepatic primary sites. The patient was a 38-year-old female. Abdominal ultrasound showed an 8 cm tumour in liver during a routine examination. Liver biopsy was done. The tumour was first considered a metastatic hepatic tumour on histopathological examination. No clues to the origin of a primary tumour were found. Upper and lower endoscopy of the GI tract and chest CT were performed to search for a primary tumour and were negative for any tumour. One month later, more extensive areas of the tumour were seen on histopathological examination of second liver biopsy with the same morphologic characteristics as the first biopsy. Immunohistochemically, there was positive staining for synaptophysin, CD 56, and S-100 in the tumour cells. These findings suggested the diagnosis of NET. The diagnosis of primary liver NET was considered in a multidisciplinary meeting. Then, left hepatectomy was performed. The final pathologic diagnosis of the tumour in the resected liver specimen was Grade II NET. The patient was doing well at postoperative 28-month follow-up.

Surgical portosystemic shunts and the Rex bypass in children: a single-centre experience.

OBJECTIVES: This study aimed to illustrate the indications for, and types and outcomes of surgical portosystemic shunt (PSS) and/or Rex bypass in a single centre. METHODS: Data were collected from children with a PSS and/or Rex bypass between 1992 and 2006 at Mount Sinai Medical Center, New York. RESULTS: Median age at surgery was 10.7 years (range 0.3-22.0 years). Indications included: (i) refractory gastrointestinal bleeding in portal hypertension associated with (a) compensated cirrhosis (n= 12), (b) portal vein thrombosis (n= 10), (c) hepatoportal sclerosis (n= 3); (ii) refractory ascites secondary to Budd-Chiari syndrome (n= 3), and (iii) familial hypercholesterolaemia (n= 4). There were 20 distal splenorenal, four portacaval, three Rex bypass, two mesocaval, two mesoatrial and one proximal splenorenal shunts. At the last follow-up (median 2.9 years, range 0.1-14.1 years), one shunt (Rex bypass) was thrombosed. Two patients had died and two had required a liver transplant. These had a patent shunt at last imaging prior to death or transplant. CONCLUSIONS: Portosystemic shunts and Rex bypass have been used to manage portal hypertension with excellent outcomes. In selected children with compensated liver disease, PSS may act as a bridge to liver transplantation or represent an attractive alternative.