Mesenchymal Hamartoma in an Adult

Posted By Maud Morshedi
Mesenchymal Hamartoma in an Adult

Clinical Summary A 27 year old woman with no significant past medical history presented with increasing right upper quadrant and right upper shoulder discomfort. Physical exam demonstrated a nontender enlarged liver. Laboratory values including serum transaminases were unremarkable. Tumor markers such as alpha-fetoprotein, carcinoembryonic antigen, human chorionic gonadotropin, and carbohydrate antigen19-9 were negative. Imaging Findings Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) studies demonstrated a large, complex cystic and solid lesion arising in the caudate lobe of the liver with major vascular involvement (Fig. 1). Contrast enhanced CT of the abdomen demonstrated that the caudate lobe of the liver was near completely replaced by the mass, which contained low attenuating no enhancing multiloculated cystic portions and enhancing solid stroma and septations (Fig. 1A-C). The main portal vein was anteriorly displaced by the tumor, the inferior vena cava was posteriorly displaced by the tumor, and the hepatic veins were seen traversing the displaced/compressed liver. The gallbladder was compressed and anteriorly displaced and contained gallstones. MRI of the abdomen again showed the caudate lobe of the liver to be near completely replaced by the mass with multiloculated nonenhancing cystic portions that were hypointense on T1-weighted images and hyperintense on T2-weighted images with progressively enhancing solid stroma and septations (Fig. 2A-F). No restricted diffusion was seen in the mass. Clinical Course/Operative Summary Given the location of the mass in the caudate lobe, ex-vivo resection with auto-transplantation of the liver was deemed the safest approach for resection by the surgeon. Initially intraoperative biopsies of the caudate lobe mass were performed and found to be nondiagnostic on frozen sections. Cold perfusion of the liver with ex-vivo caudate lobe resection containing the entirety of mass followed by hepatic auto-transplantation was then performed. A massive pseudoencapsulated solid and cystic lesion extending from the caudate lobe was seen intraoperatively, displacing the porta hepatis anteriorly and rotating the right kidney inferiorly and anteriorly (Fig. 3A). The porta hepatis was splayed open and the portal vein bifurcation was pushed anteriorly by the mass, which extended inferiorly to the level of the celiac axis. No normal liver was seen between the hepatic veins and this lesion. The hepatic veins were draped over the mass, which compressed both the IVC and hepatic veins. The hepatic artery was stretched across the lesion. The liver itself appeared normal with the exception of the caudate lesion. The IVC and hepatic veins were resected and reconstructed with additional reconstruction of the hepatic artery and portal vein as part of the autotransplantation of the liver. A choledocholedochostomy was also performed. The patient tolerated the procedure well and had a relativel

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  • Roland Talanow 2014-10-11 17:51:34

    Interesting case!

    Reply