A 5-month-old male was referred to our hospital for evaluation and management of jaundice, which began 9 weeks prior to presentation. The patient had a liver biopsy at another hospital with results suggestive of biliary atresia. The patient presented with the following laboratory values: total bilirubin, 7.5 mg dl–1; direct bilirubin, 6.1 mg dl–1; serum glutamic-oxalocetic transaminase, 253 u l–1; serum glutamic-pyruric transaminase, 127 u l–1; alkaline phosphatase, 457 u l–1; gamma-glutamyl transpeptidase, 692 u l–1; amylase, <30 u dl–1 and lipase, 225 u l–1. An ultrasound was performed that showed diffuse homogenous enlargement of the pancreatic head (Figure 1). The mass was not hypervascular on colour flow Doppler assessment. The remainder of the pancreas was normal in appearance. The pancreatic duct distal to the head was dilated. The liver parenchyma appeared heterogeneous and mild dilatation of the common bile duct and the intrahepatic biliary system was noted. Findings suggested an underlying mass in the pancreatic head, so CT and MR studies were performed.
The CT scan of the abdomen demonstrated a large and diffuse homogeneously and intensely enhancing soft tissue mass involving the head, body and uncinate process of the pancreas (). Enhancing soft tissue, which extended beyond the confines of the pancreas into the region of the porta hepatis and appeared contiguous with this mass, was also noted. The superior mesenteric vein (SMV) narrowed just prior to its junction with the splenic vein and was surrounded by this mass; therefore, involvement of the vein could not be excluded. The superior mesenteric artery (SMA) was partly encased by the mass. As noted on the ultrasound, there was dilatation of both the distal pancreatic duct and the biliary system, which suggested obstruction.
Transverse ultrasound image of the abdomen, demonstrating diffuse homogenous enlargement of the pancreatic head (arrow). The mass is not hypervascular on colour Doppler.
MRI demonstrated similar findings to those seen on the CT and ultrasound studies. A homogeneously enhancing mass was again noted involving the pancreatic head, proximal body and uncinate process (). The mass was isointense on the T1 weighted sequence and appeared slightly hyperintense on the T2 weighted sequence. The mass was found to extend into the porta hepatis following the course of the portal veins. The mass displaced the duodenum laterally and anteriorly.
Figure 2 Axial post-contrast CT scan at the level of the pancreas (a,b) a well defined intensely and homogenously enhancing mass in the head, body and uncinate process of the pancreas (short thick black arrows). Peripancreatic enhancing soft tissue is also noted (more ...)
The main diagnoses considered at this point were pancreaticoblastoma or a pancreatic haemangioma. Pancreaticoblastoma was favoured, owing to the presence of the enhancing soft tissue seen outside the pancreatic parenchyma in the region of the porta hepatis. This was thought to be either due to extension of the tumour or to associated lymphadenopathy.
The patient then underwent surgery. The mass was centred in the head of the pancreas. It was very vascular and bled upon touching. The SMA and distal SMV and splenic veins were invaded by the tumour. As a result of the extensive nature of the tumour and vascular involvement, it was decided that the only way to remove this tumour was via autotransplantation. A Whipple procedure with intestinal autotransplantation was performed. The distal splenic vein was resected and a splenorenal shunt placement was performed. The mass was sent for pathological evaluation.
Gross examination of the mass revealed an irregular firm tan-brown tumour measuring 3.2×3.2×2.9 cm involving the pancreatic head, which was surrounded by several arterial and venous blood vessels (). The tumour was locally invasive and extended to the common bile duct and mesenteric duodenal serosa and infiltrated the liver, local nerves, lymph nodes and blood vessels. On histological examination, the tumour was characterised by thin-walled vessels interspersed with coalescent nodules of spindle neoplastic cells forming small slit-like spaces filled with red blood cells, and mixed with nests of round epithelioid cells and fibrin thrombi-rich capillaries. The cells had pale cytoplasm and oval nuclei with delicate nuclear chromatin. The nuclear atypia was minimal and the rate of mitosis was low (). Neoplastic cells were positive for endothelial cell marker CD31, focally for glucose transporter 1, and negative for keratin and chromogranin. Immunostain for Ki-67 showed a high proliferative index. The final pathological diagnosis was haemangioendotheliomatosis.
Figure 3 (a) Axial and (b) coronal T1 weighted fat saturated post-contrast and axial fat saturated T2 weighted. (c) MRI of the abdomen demonstrating the large enhancing mass involving the head and uncinate process of the pancreas (long thin arrows). Peripancreatic (more ...)
Figure 4 (a) Gross cut specimen an irregular, firm, tan-brown tumour encased by the C-loop of the duodenum. The superior mesenteric artery and the superior mesenteric vein (arrows) are seen encased by the tumour. (b) Histological slide shows thin wall vessels (more ...)
The patient had a complicated post-surgery course, with thrombosis of the SMA, intestinal necrosis and thrombosis of the right superficial femoral artery. The thrombosis of the right superficial femoral artery was probably due to catheterisation. Since discharge, the patient has been doing well.