Massive pneumopritoneum with no evidence of hollow viscus perforation, a possible hepatobiliary source; a case report

Abstract

joining the left and right hepatic duct to form a single hepatico-jejunostomy and Roux-en-Y reconstruction with jejuno-jejunostomy. Operative recovery was uneventful and she was discharged routinely on day 5 post-operatively. Final histology revealed a Grade 2 pT1 pN0 Mx NET of size 17x13x16mm with invasion of the bile duct wall and Ki67 index of 15%. Immunohistochemistry showed expression of chromogranin and synaptophysin. Post-operative Ga68-DOTATATE PET scan demonstrated no evidence of tracer avid disease and therefore final diagnosis was documented as primary common hepatic duct NET. 10 months post operatively she was well with no disease recurrence. Conclusion: Here we describe a rare condition managed with a limited resection and reconstruction resulting in an R0 resection. The first reported case of a bile duct neuroendocrine tumour was in 1959 and since then there has been roughly 150 cases reported in the literature. They are exceedingly difficult to diagnose pre-operatively due to their rarity and lack of distinguishing features. The mean age of diagnosis is 47.04 AE 17.62 years (6-79 years). To our knowledge, this is only the 8th case reported in children or adolescents and the first case diagnosed with frozen section intra-operatively. Previous studies have demonstrated a possible link between GI NETs and minimal change glomerulonephritis but there is no reported literature stating a link with bile duct NETs. A systematic reviewed showed a mean follow up range of 35.28 AE 42.3 months with no reported cases of recurrence in this time.